Imaging Atlas of Ophthalmic Tumors and Diseases 303117478X, 9783031174780

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Imaging Atlas of Ophthalmic Tumors and Diseases J. Matthew Debnam

Imaging Atlas of Ophthalmic Tumors and Diseases

J. Matthew Debnam Editor

Imaging Atlas of Ophthalmic Tumors and Diseases

Editor J. Matthew Debnam Department of Neuroradiology Division of Diagnostic Imaging The University of Texas MD Anderson Cancer Center Houston, TX, USA

ISBN 978-3-031-17478-0    ISBN 978-3-031-17479-7 (eBook) https://doi.org/10.1007/978-3-031-17479-7 © The Editor(s) (if applicable) and The Author(s), under exclusive license to Springer Nature Switzerland AG 2023 This work is subject to copyright. All rights are solely and exclusively licensed by the Publisher, whether the whole or part of the material is concerned, specifically the rights of translation, reprinting, reuse of illustrations, recitation, broadcasting, reproduction on microfilms or in any other physical way, and transmission or information storage and retrieval, electronic adaptation, computer software, or by similar or dissimilar methodology now known or hereafter developed. The use of general descriptive names, registered names, trademarks, service marks, etc. in this publication does not imply, even in the absence of a specific statement, that such names are exempt from the relevant protective laws and regulations and therefore free for general use. The publisher, the authors, and the editors are safe to assume that the advice and information in this book are believed to be true and accurate at the date of publication. Neither the publisher nor the authors or the editors give a warranty, expressed or implied, with respect to the material contained herein or for any errors or omissions that may have been made. The publisher remains neutral with regard to jurisdictional claims in published maps and institutional affiliations. This Springer imprint is published by the registered company Springer Nature Switzerland AG The registered company address is: Gewerbestrasse 11, 6330 Cham, Switzerland

To my wife and best friend, Stacy, for her love, encouragement, and inspiration. To our beloved children, Celeste and Andrew: keep reaching for the stars. To my parents, Fran and Dr. James Debnam, for their education, support, and love.

Preface

The primary goal of the Imaging Atlas of Ophthalmic Tumors and Diseases is to provide a brief review of the relevant anatomy, pertinent background, and imaging features of a selection of tumors and diseases that arise in the orbit, affect vision, or may be seen in the practice of ophthalmic radiology. The sections covered in the Atlas include complex anatomic locations, including the skin and periorbital region, the globe, lacrimal gland, orbit, skull base, paranasal sinuses, pituitary gland, cavernous sinus, optic pathway, and cranial nerves. They are the origin of a multitude of pathological processes, including neoplastic, vascular, and infectious origins. The Atlas is intended for radiology residents, fellows, general radiologists, and neuroradiologists. At the same time, this Atlas may also be useful for surgeons and medical and radiation oncologists who review these imaging studies for diagnosis and treatment planning. The Atlas focuses on routine and state-of-the-art applications, primarily MRI, but also CT, PET/CT, and ultrasound, and assumes a basic knowledge of these modalities and radiologic anatomy. I have the distinct privilege of working with outstanding, dedicated faculty and fellows at The University of Texas MD Anderson Cancer Center, with whom I have had the pleasure of collaborating. These world-renowned experts include those from oculoplastic surgery, head and neck surgery, neurosurgery, and radiation oncology, as well as my colleagues in neuroradiology. They have assisted in providing “Key points” for the tumors and diseases for most of the sections and have offered additional insights upon review. These “Key points” are either a review of the clinical and imaging features of the lesions, a summary of knowledge gained from years in focused clinical practice, or pertinent information that may be requested from the radiologist in their reports. The Atlas provides an overview of the more commonly encountered and rarer tumors and tumor-like diseases seen at MD Anderson and references. Whenever possible, the presented cases either had pathologic confirmation of the diagnosis or temporal stability on follow-up imaging. Because the appearance of orbital tumors and diseases can overlap, the images in this Atlas are not an absolute confirmation of pathology. The presented descriptions and figures provide information to assist the reader in generating an appropriate differential diagnosis. The reader is also referred to multiple references throughout the Atlas for more in-depth reading to assist in the diagnosis. In summary, I hope that the Imaging Atlas of Ophthalmic Tumors and Diseases is of sufficient depth to assist radiologists and other clinicians when interpreting imaging studies of ophthalmic tumors and diseases. It is also my hope that the reader will benefit from this effort that comprises the work of many experts, a medical illustrator, editors, and the publisher. Houston, TX, USA

J. Matthew Debnam

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Contents

1 Periorbital  Skin and Eyelids �������������������������������������������������������������������������������������   1 J. Matthew Debnam and Michael E. Kupferman 2 Globe ���������������������������������������������������������������������������������������������������������������������������  43 J. Matthew Debnam, Jiawei Zhou, and Bita Esmaeli 3 Lacrimal  Gland and Nasolacrimal Drainage Apparatus ���������������������������������������  79 J. Matthew Debnam, Jiawei Zhou, and Bita Esmaeli 4 Orbit����������������������������������������������������������������������������������������������������������������������������� 119 J. Matthew Debnam, Jiawei Zhou, and Bita Esmaeli 5 Skull  Base and Bone��������������������������������������������������������������������������������������������������� 167 J. Matthew Debnam, Franco Rubino, and Shaan M. Raza 6 Sinonasal ��������������������������������������������������������������������������������������������������������������������� 213 J. Matthew Debnam, Jiawei Zhou, Bita Esmaeli, and Ehab Y. Hanna 7 Pituitary Gland����������������������������������������������������������������������������������������������������������� 255 J. Matthew Debnam, Franco Rubino, and Shaan M. Raza 8 Cavernous Sinus ��������������������������������������������������������������������������������������������������������� 279 J. Matthew Debnam, Franco Rubino, Jiawei Zhou, Bita Esmaeli, and Shaan M. Raza 9 Retrochiasmatic Optic Pathway ������������������������������������������������������������������������������� 309 J. Matthew Debnam and Nandita Guha-Thakurta 10 Cranial Nerves II–VI ������������������������������������������������������������������������������������������������� 333 J. Matthew Debnam 11 Treated Orbit��������������������������������������������������������������������������������������������������������������� 359 J. Matthew Debnam, Jiawei Zhou, and Bita Esmaeli Index�����������������������������������������������������������������������������������������������������������������������������������  389

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Editor and Contributors

Editor J. Matthew Debnam, MD  Department of Neuroradiology, Division of Diagnostic Imaging, The University of Texas MD Anderson Cancer Center, Houston, TX, USA

Contributors Bita  Esmaeli, MD  Department of Plastic Surgery, Division of Surgery, The University of Texas MD Anderson Cancer Center, Houston, TX, USA Nandita  Guha-Thakurta, MD Department of Neuroradiology, Division of Diagnostic Imaging, The University of Texas MD Anderson Cancer Center, Houston, TX, USA Ehab  Y.  Hanna, MD Department of Head and Neck Surgery, Division of Surgery, The University of Texas MD Anderson Cancer Center, Houston, TX, USA Michael  E.  Kupferman, MD, MBA  Department of Head and Neck Surgery, Division of Surgery, The University of Texas MD Anderson Cancer Center, Houston, TX, USA Shaan M. Raza, MD  Department of Neurosurgery, Division of Surgery, The University of Texas MD Anderson Cancer Center, Houston, TX, USA Franco Rubino, MD  Department of Neurosurgery, Division of Surgery, The University of Texas MD Anderson Cancer Center, Houston, TX, USA Jiawei  Zhou, MD  Department of Plastic Surgery, Division of Surgery, The University of Texas MD Anderson Cancer Center, Houston, TX, USA

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1

Periorbital Skin and Eyelids J. Matthew Debnam and Michael E. Kupferman

The most common cancers in the United States arise in the skin, the human body’s largest organ [1]. While biopsy is the standard for diagnosis of skin lesions [2], certain clinical and imaging features may assist radiologists, histopathologists, and referring clinicians in narrowing the differential diagnosis. The most common skin cancers originate in the epithelial surface and include basal cell carcinoma, followed by squamous cell carcinoma, melanoma, Merkel cell carcinoma, and sebaceous carcinoma [3]. Less commonly occurring tumors of the skin surface and subcutaneous soft tissues include various sarcomas and desmoid tumors. Lymphoma may also involve the skin surface and can be primary or secondary to disseminated disease. Complementary to the physical examination of the skin is cross-sectional imaging which may be used for diagnosis, staging, and treatment decisions for patients with skin tumors. Cross-sectional imaging is also helpful in avoiding misdiagnoses, including neurofibromas and infection. With thin-section imaging, computed tomography (CT) aids in evaluating tumors of the dermal surface, eyelids, and any bony involvement by the tumors. High-resolution magnetic resonance imaging (MRI) provides an assessment of soft tissue tumors and perineural tumor spread. Positron emission tomography (PET)/CT evaluates the metabolic activity of a tumor, provides information about local and distant metastases, determines biopsy sites based on metabolic activity, and evaluates treatment response. Ultrasound aids in detecting parotid and neck adenopathy and guiding fine-needle aspiration or core biopsy.

The purpose of this chapter is to describe the demographics and imaging appearance of common and uncommon malignancies of the skin, eyelids, and subcutaneous soft tissues. Through a review of the disease background, clinical presentation, and imaging features on various modalities, this chapter aims to provide radiologists with a means to narrow their differential diagnosis when evaluating skin malignancies.

 natomy of the Supraorbital and Infraorbital A Foramen and Perineural Tumor Spread Figure 1.1 shows the imaging appearance of the supraorbital and infraorbital foramen and perineural spread. • Knowledge of the location and normal imaging appearance of the supraorbital and infraorbital foramen is important to exclude perineural tumor spread. • The supraorbital foramen is a small opening in the superior orbital margin of the frontal bone that transmits the supraorbital branch of the ophthalmic division of the trigeminal nerve (V1). • The infraorbital foramen is a small opening in the maxillary bone located below the inferior orbital margin that transmits the infraorbital branch of the maxillary nerve (V2). • Assess for loss of normal for fat and widening or destruction of the bony foramen.

J. M. Debnam (*) Department of Neuroradiology, Division of Diagnostic Imaging, The University of Texas MD Anderson Cancer Center, Houston, TX, USA e-mail: [email protected] M. E. Kupferman Department of Head and Neck Surgery, Division of Surgery, The University of Texas MD Anderson Cancer Center, Houston, TX, USA e-mail: [email protected] © The Author(s), under exclusive license to Springer Nature Switzerland AG 2023 J. M. Debnam (ed.), Imaging Atlas of Ophthalmic Tumors and Diseases, https://doi.org/10.1007/978-3-031-17479-7_1

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J. M. Debnam and M. E. Kupferman

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Fig. 1.1  Supraorbital and infraorbital foramen. (a) Axial CT with contrast, bone window shows the left supraorbital foramen (arrow). (b) Axial CT with contrast, bone window shows the right infraorbital foramen (arrow). (c–f) A 70-year-old male with right premaxillary melanoma and perineural tumor spread. (c) Axial CT with contrast, bone window shows widening of the right infraorbital foramen (arrow) with adjacent destruction of the maxillary bone. Note the normal appearance of the left infraorbital foramen (thin arrow). (d) Axial CT with contrast, soft tissue window shows tumor in the wid-

ened right infraorbital foramen and replacement of the normal foraminal fat (arrow). Note the normal appearance of fat in the left infraorbital foramen (thin arrow). (e) Axial T1 non-contrast MRI without fat saturation shows melanoma replacing normal right infraorbital foraminal fat (arrow). Note the normal fat near the left infraorbital foramen (thin arrow). (f) Coronal T1 post-contrast MRI with fat saturation shows perineural spread along the right infraorbital nerve (arrow)

Basal Cell Carcinoma

• Local spread occurs along the periosteum, perichondrium, and fascia [5]. • About 2–25% recur locally, depending on the completeness of excision [6]. • Basal cell nevus syndrome (Gorlin syndrome) is a rare autosomal dominant disorder characterized by multiple BCCs with coarse calcification of the falx cerebri, sellar bridging (fusion of anterior and posterior clinoid processes), skeletal malformations, and odontogenic keratocysts. –– These patients are predisposed to several malignancies including melanoma, non-Hodgkin’s lymphoma, meningiomas, medulloblastoma, breast cancer, and ovarian fibromas [7–9].

Figures 1.2, 1.3, and 1.4 show cases of basal cell carcinoma.

Background • The most common non-melanomatous skin cancer is basal cell carcinoma (BCC). • Slow-growing neoplasm occurs approximately three times more often than squamous cell carcinoma (SCC) [4]. • Approximately 85–93% occur in the head and neck [5].

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Fig. 1.2  A 65-year-old female with a slowly growing basal cell carcinoma of the left lower eyelid. (a, b) Axial and coronal T1 post-contrast MRI shows a small enhancing nodule on the left lower eyelid (arrows)

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Fig. 1.3  A 65-year-old female with recurrent basal cell carcinoma of the right lower eyelid. (a) Axial T1 non-contrast MRI without fat saturation shows an isointense lesion in the right lower eyelid and periorbital soft tissues (white arrow) with extension into the inferior orbit beneath the globe (black arrow). (b) Axial T1 post-contrast MRI

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with fat saturation shows homogeneous enhancement of the lesion (arrows). (c) Axial T2 MRI with fat saturation shows a rounded enhancing right parotid gland node (arrow) that was subsequently biopsied as metastatic basal cell carcinoma

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Fig. 1.4  A 38-year-old female with Gorlin syndrome and a left supraorbital basal cell carcinoma. (a) Axial CT with contrast, soft tissue window shows an enhancing subcutaneous soft tissue mass adjacent to the left superior orbital rim (white arrow) with subtle extension into

the superior orbit (black arrow). (b) Axial CT with contrast, bone window shows multiple coarse calcifications along the dura and falx cerebri (arrows)

Presentation

MRI

• BCC is more common in men with light skin and usually arises in the central midface and eyelids [5, 10]. • BCC appears as a small round or oval nodule on the skin surface that may ulcerate [5, 11].

• T1 hypointense compared to muscle, heterogeneously T2 hyperintense, and enhances [4].

Imaging

• Lesions are FDG-avid on 18F-FDG PET/CT [14].

• BCC involves the dermal surface and may extend into the subcutaneous soft tissues while larger lesions may show signs of infiltration. • BCC rarely metastasizes (90%: may be single, multiple, or of varied sizes and shapes [17, 18].

MRI • T1 hyperintense to vitreous, scattered areas of hypointensity corresponding to calcifications. • T2 hypointense to the vitreous. • Moderate to marked enhancement [7, 9, 19–21].

Key Points

Medulloepithelioma Figure 2.7 shows a case of a medulloepithelioma.

Background • Second most common tumor of the eye in children. • Characteristically arises in the neuroectoderm of non-­ pigmented ciliary body epithelium [23]. • May also arise from the retina, choroid, or inner lining of the optic nerve [24]. • Teratoid and non-teratoid types. • Teratoid type has additional hyperplastic elements including cartilage and skeletal muscle.

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Fig. 2.7  A 10-month-old child with glaucoma and a medulloepithelioma in the left globe seen on ophthalmologic exam. (a) Axial T1 non-­ contrast MRI without fat saturation shows a hyperintense mass involving the left ciliary body (arrow). (b) Axial T2 MRI with fat saturation demonstrates that the mass is hypointense (white arrow). There

is also a retinal detachment (black arrow) and soft tissue thickening in the lateral orbit (thin arrow) related to a glaucoma shunt. (c) Axial T1 post-contrast MRI with fat saturation shows enhancement of the left ciliary body medulloepithelioma (arrow). Soft tissue thickening in the lateral orbit (thin arrow) is related to the glaucoma shunt

• Tumor confined to the globe has a favorable prognosis. • Extra-scleral extension increases recurrence and metastasis rate resulting in poor survival [25, 26].

MRI

Presentation • Mean age at occurrence is 5 years old; most lesions are diagnosed before age 10 [5]. • Usually unilateral. • No known risk factors; no gender or race predilections [23, 24]. • Symptoms: ocular pain, loss of visual acuity that may be attributed to lens subluxation, cataract formation, and secondary glaucoma. • Less common complications: retinal detachment, posterior chamber hemorrhage [27].

T1 isointense to slightly hyperintense. T2 hypointense to vitreous. Avid enhancement [23, 26]. Soft tissue may extend posteriorly along the ciliary body. • May have intratumoral cysts and abnormal signal in the vitreous [28]. • • • •

Key Points • Search for spread outside the globe and for metastases.

Nevus

Imaging

Figures 2.8 and 2.9 show cases of a nevus.

• Solid enhancing mass of variable size. Larger tumors may have cystic components.

Background

CT • Intense enhancement. • Normal or slightly hyperdense vitreous may be present [26]. • Calcification of heteroplastic hyaline cartilage is rarely seen.

• Congenital lesion usually detected in the first decade [29]. • Present in approximately 6% of White population [30]. • Rarely evolve into malignant melanoma [31]; risk increases with age [32]. • Patients with asymptomatic choroidal nevi should initially be monitored twice yearly with annual follow-up thereafter if the lesion is stable [33].

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Fig. 2.8  An 80-year-old male with a right choroidal nevus. (a) Axial T2 MRI without fat saturation shows a lenticular hypointense mass involving the right choroid near the optic nerve head (arrow). (b)

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Axial FLAIR MRI with fat saturation shows signal hyperintensity of the nevus (arrow). (c) Sagittal T1 post-contrast MRI without fat saturation shows enhancement of the lenticular-shaped nevus (arrow)

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Fig. 2.9  An 81-year-old female with a right choroidal nevus. (a) Axial non-contrast enhanced MRI without fat saturation shows a lenticular-­ shaped hyperintense nevus on the right choroid (arrow). (b) Axial

T2 MRI with fat saturation demonstrates that the mass is hypointense (arrow). (c) Axial T1 post-contrast MRI with fat saturation shows enhancement of the lenticular-shaped nevus (arrow)

Presentation

CT and MRI

• Usually discovered on routine dilated fundus examination; most patients are asymptomatic. • Symptoms: decreased vision, visual field defect, flashes, and floaters [33].

• Similar to choroidal melanoma with T1 hyperintensity, T2 hypointensity, and enhancement.

Imaging

• Careful search of the globe with thin section imaging may be required for diagnosis. • If a nevus is followed clinically, search for an increase in size and spread outside the globe, hemorrhage, and retinal detachment.

• Most common location is the posterior choroid. • Lentiform shape with a thickness of less than 2  mm [30].

Key Points

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Angioma (Hemangioblastoma)

• Usually detected in adolescence and early adulthood; those associated with VHL are detected earlier [35]. • Other lesions of VHL: CNS hemangioblastomas, pancreatic tumors, pheochromocytomas, renal cysts, and clear cell carcinoma, epididymal cysts, cystadenomas of the broad ligament, and endolymphatic sac tumors.

Figure 2.10 shows a case of an angioma.

Background • Benign vascular hamartoma. • May occur as an isolated lesion or associated with phakomatosis (VHL) [34]. • Of patients 1.6  cm, location within the ciliary body, extension beyond the globe, male gender, and rapidly growing tumors [69].

Presentation • Most often affects Whites [70]. • Higher incidence noted with increasing age; only 2% occur in patients 50% of cases). • Non-traumatic lens dislocation etiologies: connective tissue disorders, including Marfan syndrome, Ehlers-­ Danlos syndrome, and homocystinuria [117]. • Bilateral lens dislocation suggests a systemic condition.

CT • A shrunken globe has linear or mottled calcification of devitalized tissue. • Scarring can cause an irregular shape of the globe [116].

Presentation

MRI

• Symptoms: poor vision, glaucoma, uveitis, retinal detachment, and chorioretinal degeneration.

• The globe appears T1 isointense with heterogeneous areas of signal hyperintensity related to hemorrhage or calcification. • On T2-weighted imaging the vitreous is heterogeneous with “filling defects” from calcifications. • FLAIR sequence shows hyperintense signal relative to the normal hypointense signal of the globe.

Key Points • Should not be mistaken for an ocular prosthesis.

Ocular Lens Displacement

Imaging • Following a complete posterior subluxation, the lens lies dependently in the vitreous humor. • If zonular fibers are partially disrupted, the intact fibers will hold one margin of the lens in a normal position behind the iris while the portion of the lens with disrupted fibers will be angled posteriorly in the vitreous humor.

Key Points • May mimic a choroidal melanoma metastasis [117, 118].

Figure 2.34 shows cases of ocular lens displacement.

Fig. 2.34  Ocular lens displacement. (a) Axial CT without contrast, soft tissue window demonstrates posterior displacement of the right lens (arrow) from the normal position as illustrated in the left globe (thin arrow). (b) Axial T2 MRI without fat saturation in a different patient without fat saturation shows bilateral lens displacement

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Scleritis

Imaging

Figure 2.35 shows a case of scleritis.

• Scleral thickening and enhancement, periscleral cellulitis [119]. • Indirect signs: retinal and choroidal detachment with effusion involving the suprachoroidal space and uveitis [119, 121].

Background • Rare condition that threatens vision and can occur in isolation or associated with other abnormalities of the orbit. • Etiology is usually a noninfectious inflammatory condition that is either idiopathic or related to systemic diseases. –– Most common etiologies are Rheumatoid arthritis and granulomatosis with polyangiitis [119]. • Anterior scleritis is more common and can be diagnosed on direct inspection without imaging [120]. • Posterior scleritis (2–12% of cases). –– Often underdiagnosed owing to rarity and variable signs and symptoms [121].

CT • Eccentric wall-thickening of the globe with peripheral enhancement.

MRI • Scleral enhancement may be the only finding. • Normal T1 signal hyperintensity of fat can mask scleral enhancement; fat saturation should be used [119].

Presentation

Key Points

• Orbital pain in 60% of patients [119]. • Other symptoms: headache, decreased and permanent loss of vision [122]. • Fever may be present from infectious scleritis [120].

• Scleral enhancement is an abnormal finding and should not be confused with physiologic choroidal enhancement.

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Fig. 2.35  A 69-year-old man with uveitis and scleritis complicated by a serous retinal detachment. (a) Axial T1 post-contrast MRI with fat saturation shows enhancement of the outer aspect of the right sclera (arrow), right uvea, and lacrimal gland (thin arrows). (b) Coronal T1 post-contrast MRI with fat saturation shows enhancement of the outer

aspect of the right sclera (white arrow), right uvea, and lacrimal gland (thin arrows) with an associated choroidal detachment (black arrow). (c) Axial T2 MRI with fat saturation demonstrates enlargement of the right lacrimal gland (arrow) and serous right choroidal detachments (thin arrows)

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Drusen

Imaging

Figure 2.36a shows a case of drusen.

• Most optic disc drusens are calcified and vary in size from >1 mm to 4 mm in diameter by 3 mm in thickness [126].

Background • Deposition of mucopolysaccharides and proteinaceous material [123]. • Acellular concretions that accumulate in the optic nerve head [124].

Presentation • • • • •

Occurs in up to 2.0% of the population. More common in Whites [125]. Usually asymptomatic and discovered incidentally. May mimic papilledema fundoscopically [126]. Visual field defects may develop or rarely loss of central visual acuity may occur from the development of CNV (growth of new vessels originating from the choroid and extending into the sub-retinal pigment epithelium or subretinal space) [127].

CT • In childhood, drusen is small and not mineralized. With thin-section imaging, small regions of hyperdensity and swelling may be present. • In the adult population, drusen appears as small, well-­ defined calcifications [124].

MRI • A T2 hypointense filling defect may be present. • No enhancement.

Key Points • Metallic foreign bodies with have higher Hounsfield unit measurements.

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Fig. 2.36 (a) Drusen: Axial CT with contrast, soft tissue window shows benign calcification at the optic nerve heads (arrows). (b) Senile calcification: Axial CT non-contrast, soft tissue window shows calcification of the sclera (arrows). (c) Trochlear calcification: Axial

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CT with contrast, bone window demonstrates calcification of the trochlear apparatus bilaterally (arrows). The trochlear apparatus is a cartilaginous structure in the superonasal orbit that permits movement of the superior oblique tendon

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Senile Calcification Figure 2.36b shows a case of senile calcification.

Background • Benign calcific depositions in the scleral stroma are a common incidental finding [128].

Presentation • Incidence increases with age and is common in the elderly. • Asymptomatic.

Imaging • Occur about 2  mm anterior to the insertion of the medial and lateral rectus muscles upon the globe. • Calcifications involving the vertical rectus muscle insertions are rare.

CT • Calcifications of variable size may be single or multiple and unilateral or bilateral [128].

Key Points Senile and trochlear apparatus calcifications should not be mistaken for metallic foreign bodies.

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J. M. Debnam et al. 109. Yan X, Edward D, Mafee M.  Ocular calcification: radiologic-­ pathologic correlation and literature review. Int J Neuroradiol. 1998;4:81–95. 110. DePotter P, Shields JA, Shields CL, Rao VM. Magnetic resonance imaging in choroidal osteoma. Retina. 1991;11:221–3. https://doi. org/10.1097/00006982-­199111020-­00006. 111. Reiter MJ, Schwope RB, Kini JA, York GE, Suhr AW. Postoperative imaging of the orbital contents. Radiographics. 2015;35:221–34. https://doi.org/10.1148/rg.351140008. 112. Hallinan JT, Pillay P, Koh LH, Goh KY, Yu WY. Eye globe abnormalities on MR and CT in adults: an anatomical approach. Korean J Radiol. 2016;17(5):664–73. https://doi.org/10.3348/kjr.2016.17.5.664. 113. LeBedis CA, Sakai O. Nontraumatic orbital conditions: diagnosis with CT and MR imaging in the emergent setting. Radiographics. 2008;28:1741–53. https://doi.org/10.1148/rg.286085515. 114. Roy AA, Davagnanam I, Evanson J.  Abnormalities of the globe. Clin Radiol. 2012;67:1011–22. https://doi.org/10.1016/j. crad.2012.03.006. 115. Tripathy K, Chawla R, Temkar S, Sagar P, Kashyap S, Pushker N, et al. Phthisis Bulbi—a clinicopathological perspective. Semin Ophthalmol. 2018;33:788–803. https://doi.org/10.1080/08820538 .2018.1477966. 116. Midyett FA, Mukherji SK.  Orbital imaging. Philadelphia, PA; 2015. p. 29–31. ISBN 978-0-323-34037-3. 117. Mason JO 3rd, Patel SA. Traumatic lens subluxation presenting as pseudomelanoma. Ophthalmic Surg Lasers Imaging Retina. 2014;45:328–30. https://doi.org/10.3928/23258160-­20140605-­02. 118. Seigel RS, Sell J, Magnus DE. CT appearance of traumatic dislocated lens. AJNR Am J Neuroradiol. 1988;9:390. 119. Diogo MC, Jager MJ, Ferreira TA.  CT and MR imaging in the diagnosis of scleritis. AJNR Am J Neuroradiol. 2016;37:2334–9. https://doi.org/10.3174/ajnr.A4890. 120. Okhravi N, Odufuwa B, McCluskey P, Lightman S.  Scleritis. Surv Ophthalmol. 2005;50:351–63. https://doi.org/10.1016/j. survophthal.2005.04.001. 121. Biswas J, Mittal S, Ganesh SK, Shetty NS, Gopal L.  Posterior scleritis: clinical profile and imaging characteristics. Indian J Ophthalmol. 1998;46:195–202. 122. Benson WE. Posterior scleritis. Surv Ophthalmol. 1988;32:297– 316. https://doi.org/10.1016/0039-­6257(88)90093-­8. 123. Donaldson L, Margolin E.  Approach to patient with unilateral optic disc edema and normal visual function. J Neurol Sci. 2021;424:117414. https://doi.org/10.1016/j.jns.2021.117414. 124. Palmer E, Gale J, Crowston JG, Wells AP. Optic nerve head drusen: an update. Neuroophthalmology. 2018;4:367–84. https://doi. org/10.1080/01658107.2018.1444060. 125. Rosenberg MA, Savino PJ, Glaser JS.  A clinical analysis of pseudopapilledema. I.  Population, laterality, acuity, refractive error, ophthalmoscopic characteristics, and coincident disease. Arch Ophthalmol. 1979;97:65–70. https://doi.org/10.1001/archo pht.1979.01020010005001. 126. McNicholas MM, Power WJ, Griffin JF. Sonography in optic disk drusen: imaging findings and role in diagnosis when funduscopic findings are normal. AJR Am J Roentgenol. 1994;162:161–3. https://doi.org/10.2214/ajr.162.1.8273656. 127. Rotruck J.  A review of optic disc drusen in children. Int Ophthalmol Clin. 2018;58:67–82. https://doi.org/10.1097/ IIO.0000000000000236. 128. Alorainy I.  Senile scleral plaques: CT.  Neuroradiology. 2000;42:145–8. https://doi.org/10.1007/s002340050035.

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Lacrimal Gland and Nasolacrimal Drainage Apparatus J. Matthew Debnam, Jiawei Zhou, and Bita Esmaeli

Lesions of the lacrimal gland usually present as a palpable mass in the superotemporal orbit. Of these, approximately 50% are inflammatory and the other 50% represent various types of tumors [1]. Lacrimal gland tumors occur in approximately 1 in one million people each year [2, 3]. More than half of the lacrimal gland tumors are epithelial in origin. Of the epithelial tumors, about 50–60% are benign and include pleomorphic adenoma and oncocytoma. The other 40–50% are malignant [2, 4], with approximately 90% being adenoid cystic carcinoma and adenocarcinoma [5]. Another one-third of lacrimal tumors are lymphoid lesions. The remaining 10–15% are mesenchymal, arise from distant metastases, or occur via direct extension from another site [6]. Epithelial tumors account for the majority of nasolacrimal drainage apparatus (NLDA) tumors, followed by mesenchymal tumors, lymphoid lesions, melanoma and other lesions including infection [7]. Unless suggested otherwise by the clinical and radiographic features, most lacrimal gland and lacrimal duct apparatus lesions undergo biopsy for diagnosis. The exception is the pleomorphic adenoma which is removed en bloc to avoid recurrence. The role of the radiologist is to assess the imaging appearance of the lacrimal lesion, which may aid in narrowing the differential diagnosis. Equally important is to comment on benign vs. malignant radiographic features and to describe the pattern and extent of disease spread, including outside of the orbit and involvement of the head and neck region. Specific imaging characteristics to mention in the radiology report for lacrimal gland tumors include the size of the

lesion, the presence of bone remodeling or destruction of the lacrimal fossa, involvement of the extraocular muscles, and/ or extension into the intraconal space, and if the disease spreads beyond the midline of the orbit, which has implications for ocular toxicity from adjuvant radiation therapy. For NLDA tumors, the report should include the size and extent of the tumor, whether the tumor extends to involve the upper or lower eyelids or the paranasal sinuses, and whether or not the cribriform plate is involved. The imaging modalities used for the evaluation of the lacrimal gland and NLDA lesions include CT, MRI, and PET/ CT.  These modalities provide important information about staging, pre-surgical planning, and treatment response. CT aids in the delineation of tumor extent and bone remodeling or destruction. MRI can also be used to evaluate the features of the tumors, including soft tissue characteristics, sinonasal and intracranial involvement, and perineural spread. PET/CT is used for measuring a tumor’s metabolic activity, detecting local and distant metastases, staging, determining a site for biopsy based on metabolic activity, and evaluating treatment response. The purpose of this chapter is to describe the demographics and imaging appearance of common and uncommon malignancies of the lacrimal gland and the NLDA. This is accomplished with a review of the disease background, clinical presentation, and imaging features on various modalities. This chapter should provide the radiologist with a means to narrow their differential diagnosis when evaluating malignancies of the lacrimal gland and nasolacrimal duct. Tumors arising from the orbit, bone and skull base, and the sinonasal cavity are discussed in other chapters.

J. M. Debnam (*) Department of Neuroradiology, Division of Diagnostic Imaging, The University of Texas MD Anderson Cancer Center, Houston, TX, USA e-mail: [email protected] J. Zhou · B. Esmaeli Department of Plastic Surgery, Division of Surgery, The University of Texas MD Anderson Cancer Center, Houston, TX, USA e-mail: [email protected]; [email protected] © The Author(s), under exclusive license to Springer Nature Switzerland AG 2023 J. M. Debnam (ed.), Imaging Atlas of Ophthalmic Tumors and Diseases, https://doi.org/10.1007/978-3-031-17479-7_3

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Anatomy Figures 3.1 shows a schematic illustration and images of the lacrimal gland and NLDA.

•

• The function of the lacrimal gland is to produce and drain tears. • Comprised of two divisions, secretory and drainage. • The secretory division consists of the lacrimal gland, which is located along the superotemporal orbital roof. • The lacrimal gland is divided into orbital and palpebral lobes by the aponeurosis of the levator palpebrae superioris. –– Larger orbital lobe located posterior and superior to the aponeurosis.

•

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• • • •

–– Palpebral lobe located anterior and inferior to the aponeurosis. Six to twelve secretory ducts drain into the lateral portion of the superior fornix on the conjunctiva of the upper eyelid. One or two lacrimal ducts may drain into the lateral portion of the inferior fornix. The drainage division consists of the NLDA. Begins with small openings (punta) located in the medial upper and lower eyelids. Two ducts (superior and inferior canaliculi) drain into the lacrimal sac. The nasolacrimal duct extends inferiorly from the lacrimal sac to empty into the inferior meatus of the nose.

Upper fornix Lacrimal gland Excretory duct to upper fornix Main gland Execretory ducts

Everted upper punctum Upper canaliculus Lacrimal sac

To lower fornix (two) Lateral lacrimal lake Lower fornix Lower everted punctum Lower canaliculus

Inferior turbinate Inferior meatus of nose

Nasolacrimal duct Nasal cavity

Fig. 3.1  Lacrimal gland and NLDA. (a) Schematic drawing of the anatomy of the lacrimal gland, ducts, and the NLDA. (From Ansari and Nadeem [8]; with permission). (b) Coronal T1 post-contrast MR with saturation shows a hyperintense appearance of bilateral lacrimal

glands located along the superotemporal orbital roof (arrows). (c) Axial CT without contrast, bone window shows the nasolacrimal ducts (arrows), which connect the lacrimal sac and the nasal cavity

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Fig. 3.1 (continued)

Lacrimal Gland Lesions

Imaging

Pleomorphic Adenoma

• Well-circumscribed and homogeneously enhancing mass in the lacrimal fossa. • Large lesions can have a heterogeneous appearance due to cystic degeneration, necrosis, or hemorrhage [11]. • Due to slow growth there may be smooth, concave bone remodeling of the lacrimal fossa. • Erosion of bone is rare [10, 11].

Figures 3.2, 3.3, and 3.4 show cases of lacrimal pleomorphic adenomas.

Background • Also named benign mixed tumor; contains epithelial elements [9]. • Accounts for approximately 57% of epithelial lesions. • Most common benign tumor involving the lacrimal gland [10].

Presentation • Manifests in the fourth or fifth decades of life. • Slow-growing tumor [11]. • Mass effect upon globe with downward displacement and proptosis. • Pain is rare. Presence of pain suggests an alternate diagnosis [12].

CT • Usually isodense (96.2%) with regular margins (94.2%) [13]. • May have calcifications.

MRI • Smaller pleomorphic adenomas appear homogeneously T1 hypointense and T2 iso- to hyperintense. • Larger lesions with hemorrhage or necrosis show variable signal intensities on T1 and T2 sequences [10].

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Fig. 3.2  A 53-year-old female with left proptosis due to a pleomorphic adenoma. (a) Axial T1 non-contrast MRI without fat saturation shows a well-circumscribed isointense pleomorphic adenoma in the superotemporal left orbit (arrow). (b) Axial T2 MRI with fat saturation shows an iso- to hyperintense appearance of the mass (arrow). (c)

Axial T1 post-contrast MRI with fat saturation shows homogeneous enhancement of the well-circumscribed, lobulated mass (arrow). (d) Coronal T1 non-contrast MRI without fat saturation shows remodeling of the left orbital roof by the pleomorphic adenoma (arrow)

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Fig. 3.3 (a, b) A 66-year-old female with progressive downward displacement of the right eye from a pleomorphic adenoma. (a) Axial CT with contrast, soft tissue window shows an isodense pleomorphic adenoma involving the right lacrimal gland (arrow). (b) Coronal CT with contrast, soft tissue window shows downward displacement of the right globe with flattening of the superior surface (white arrow).

Remodeling of the right orbital roof (black arrow) is also present. (c, d) A 70-year-old female with diplopia. (c) Axial T2 MRI with fat saturation shows a heterogeneous hypo- to hyperintense right lacrimal gland pleomorphic adenoma (arrow). (d) Axial T1 post-contrast MRI with fat saturation shows homogeneous enhancement of the well-­ circumscribed right lacrimal mass (arrow)

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Fig. 3.4  A 57-year-old male with left proptosis due to a pleomorphic adenoma. (a) Axial CT with contrast, soft tissue window shows calcifications in the left lacrimal gland pleomorphic adenoma (arrow). (b) Axial T2 MRI with fat saturation shows a heterogeneous left lacrimal gland mass with signal hypointensity from hemorrhage and/or cal-

cification (arrow) and a hyperintense cystic component (thin arrow). (c) Axial T1 post-contrast MRI with fat saturation shows heterogeneous enhancement of the solid portions of the left lacrimal mass (arrow) without enhancement of the cystic component (thin arrow)

Key Points

Presentation

• If suspected, suggest the presence of a pleomorphic adenoma so that it can be excised en bloc by the surgeon. • A biopsy for diagnosis may result in recurrence, as the 5-year recurrence rate is up to 30% [14].

• Tends to occur in adulthood at a mean age of 66 years old (range, 44–82 years) [17]. • Lesions involving the caruncle have a female predominance. • No gender predilection for the other sites of orbital oncocytic neoplasms. • Lesions tend to grow slowly and tend to be a single lesion and unilateral [17]. • Symptoms: asymptomatic or symptoms ranging from lid swelling to severe pain and proptosis [18].

Oncocytic Neoplasms See Fig. 3.5 for a case of an oncocytic neoplasm.

Background • Epithelial neoplasm arising from ductal cells. • Prominently composed of cells with an eosinophilic granular cytoplasm resulting from a large number of atypical mitochondria [15, 16]. • Categorized as oncocytic hyperplasia, oncocytic adenoma (oncocytoma), or oncocytic carcinoma, depending on the histologic features [15]. • Approximately 60% involve the caruncle (small, globular nodule in the inner corner of the eye), 20% are in the lacrimal sac, and 6% are in the lacrimal gland [17]. • Also occur in salivary, thyroid, and parathyroid glands, as well as the breast, kidney, buccal mucosa, pharynx, and larynx [18].

Imaging • Typically appear as a well-circumscribed, oval, homogeneously enhancing mass [17]. • Bone remodeling may be observed in slow-growing benign tumors [17, 19]. • Oncocytic carcinomas may exhibit aggressive features such as invasion of the extraocular muscle and bone erosion [20] and can metastasize [21].

CT • Hyperdensity on non-contrast CT [19, 22].

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Fig. 3.5  A 37-year-old female with diplopia due to an oncocytoma. (a) Coronal CT with contrast, soft tissue window shows an iso- to hyperdense appearance of the left lacrimal gland oncocytoma (arrow). Note inferior displacement of the left globe (thin arrow) due to the mass. (b) Axial CT with contrast, bone window shows remodeling of the adjacent left lateral orbital wall (arrow). (c) Coronal T2 MRI with

fat saturation shows a heterogeneous mass with multiple linear hypointense foci suggesting vascular flow voids (arrow). (d) Axial T1 post-contrast MRI with fat saturation shows homogeneous enhancement of the mass and multiple linear and punctate hypointense foci suggesting vascular flow voids (arrow)

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MRI

Adenoid Cystic Carcinoma

• Variable appearance. T1 isointense and T2 heterogeneously iso- to hyperintense [19, 22]. • Homogeneous enhancement [17, 22]. • Multiple vascular flow voids may be present [17].

Figures 3.6, 3.7, 3.8, 3.9, and 3.10 show cases of adenoid cystic carcinoma.

Key Points

• Most common lacrimal gland malignancy and second most common epithelial lesion. • Accounts for approximately 29% of lacrimal epithelial tumors and 5% of all primary orbital tumors [23].

• Oncocytic adenomas have benign features, while oncocytic carcinomas are aggressive.

Fig. 3.6  A 35-year-old male with left periorbital spasms and an adenoid cystic carcinoma. (a) Coronal T2 MRI with fat saturation shows a heterogeneous hypo- to hyperintense left lacrimal gland adenoid cystic carcinoma (arrow). (b) Axial T1 post-contrast MRI with fat saturation shows fairly homogeneous enhancement of the left lacrimal mass (arrow). Pleomorphic adenoma could have a similar appearance

Background

a

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Fig. 3.7  A 54-year-old male with irritation of the left eye due to an adenoid cystic carcinoma. (a) Axial T2 MRI without fat saturation shows a heterogeneous hypo- to hyperintense left lacrimal gland mass with curvilinear signal hypointensity related to hemorrhage

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(arrow). (b) Axial T1 post-contrast MRI with fat saturation shows fairly homogeneous enhancement of the left lacrimal adenoid cystic carcinoma (arrow). Note enhancement of the orbital dura extending posteriorly from the tumor (thin arrow)

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Fig. 3.8  A 31-year-old male with left eyelid swelling and proptosis due to an adenoid cystic carcinoma. (a) Axial T1 post-contrast MRI with fat saturation shows fairly homogeneous enhancement of the left lacrimal adenoid cystic carcinoma (arrow). (b) Coronal T1 post-­ contrast MRI with fat saturation shows extension of tumor through

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the orbital roof into the inferior frontal bone (arrow). (c) Axial T1 post-contrast MRI with fat saturation shows thickened left frontal dura with enhancement related to intracranial extension of the tumor (arrow)

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Fig. 3.9  A 41-year-old male with gradually increasing right orbital pain due to an adenoid cystic carcinoma. (a) Axial CT with contrast, bone window shows destruction of the right lateral orbital wall (arrow). (b) Axial T1 post-contrast MRI with fat saturation shows extra-orbital extension of the adenoid cystic carcinoma with involve-

ment of the right masticator space, sphenoid bone, and adjacent dural enhancement related to intracranial extension (arrows). (c) Axial 18F-FDG PET/CT shows an FDG-avid appearance of the mass (arrow)

Presentation

• With advanced disease, ACC has irregular borders, distortion of the orbital contents, including the globe, and bone erosion [10, 11]. • The cranial nerves, especially the lacrimal branch of the ophthalmic nerve (V1), should be scrutinized for evidence of perineural tumor spread [10, 11].

• Most present in the fourth decade with orbital pain [11]. • Poor prognosis [10].

Imaging • Infiltrative lesion with a strong propensity for perineural spread [11]. • Initially, lesions can be indistinguishable from a pleomorphic adenoma.

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Fig. 3.10  A 29-year-old male with left lacrimal adenoid cystic carcinoma treated at an outside institution before referral. (a) Axial T1 post-­ contrast MRI with fat saturation shows a left lacrimal mass filling the orbit with spread through the superior orbital fissure (arrow) into the middle cranial fossa. Note mass effect upon the globe with proptosis. (b) Axial T1 non-contrast MR without fat saturation shows a mass replacing the fat in the left pterygopalatine fossa from peri-

neural tumor spread (arrow). (c) Axial T1 post-contrast MRI with fat saturation shows perineural tumor spread in the left pterygopalatine fossa, left Meckel’s cave and trigeminal nerve root (arrows). (d) Axial T1 post-contrast MRI with fat saturation shows enhancement along the left mandibular nerve (V3) from perineural tumor spread (arrow)

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CT

Imaging

• Hyperdense with irregular borders and homogeneous enhancement. • Cystic changes, calcification, perineural invasion, and adjacent bony erosion may be present [10, 24, 25].

• Lesions may be round or elongated soft tissue masses, sometimes with irregular margins. • Frequently misdiagnosed as benign in nature [31].

MRI

MRI

• T1 homogeneously hypointense enhancement. • T2 hypo- to hyperintense [10].

with

moderate

Key Points

PET/CT •

• T1 hypointense, T2 iso- to hyperintense and solid to heterogeneous enhancement [32].

F-FDG avid although reported lower uptake compared to squamous cell carcinoma [26]. 18

• High rate of local recurrence. • Local and distant metastases.

Key Points

Mucoepidermoid Carcinoma

• Initially, lesions can be indistinguishable from a pleomorphic adenoma. • Describe orbital wall and intracranial involvement and disease spread through the superior orbital fissure. • Careful search of cranial nerves for evidence of perineural spread.

Figures 3.13 shows a case of lacrimal mucoepidermoid carcinoma.

Background

• Uncommon and accounts for approximately 10% of primary lacrimal gland epithelial malignancies [27]. • Aggressive lesion with a high rate of local metastasis.

• Rare malignant epithelial tumor. • Mucoepidermoid carcinoma (MEC) is usually composed of an admixture of epidermoid cells, small basal cells with a basophilic cytoplasm, larger cells with an eosinophilic cytoplasm, and mucus-secreting cells [33]. • Graded histologically as low, intermediate, or high-­ grade depending on cellular features including mitotic figures, atypical nuclei, and necrosis [34]. • Fusion oncogene CRTC1-MAML2 is present in up to 80% of cases. –– May positively impact prognosis [35]. • Can occur in the lacrimal gland or lacrimal sac [36].

Presentation

Presentation

• Mean age at presentation is 57  years and more common in males [28]. • Symptoms: pain, exophthalmos, pseudoptosis (apparent but not actual eyelid drooping), dystopia (malpositioning of the eyes), and decreased visual acuity [29]. • Patients may be asymptomatic, which leads to diagnosis at an advanced stage. • Local and distant metastases and a high rate of local recurrence [27, 30].

• Patients may present with a slow-growing painless mass and proptosis. • Other signs include swelling, decreased motility, and diplopia [35, 37].

Adenocarcinoma Figures 3.11 and 3.12 show cases of lacrimal adenocarcinoma.

Background

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Fig. 3.11  A 49-year-old female with eye irritation due to a lacrimal adenocarcinoma. (a) Axial T2 MRI with fat saturation shows heterogeneous hypo- to isointense left lacrimal gland adenocarcinoma (arrow). (b) Axial T1 post-contrast MRI with fat saturation shows peripheral enhancement of the left lacrimal mass (arrow). (c)

Coronal T1 post-contrast MRI with fat saturation shows metastatic left parotid and neck nodes (arrows). (d) Coronal T1 post-contrast MRI with fat saturation shows a left facial metastasis abutting the maxillary sinus (arrow)

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Fig. 3.12  A 51-year-old male with a “bump” in the left upper eyelid due to a poorly differentiated adenocarcinoma. (a) Axial T1 post-­ contrast MRI with fat saturation shows an enhancing left lacrimal gland mass with irregular margins and extension into the left supra-

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orbital soft tissues (arrows). (b) Coronal T1 post-contrast MRI with fat saturation shows an infiltrating tumor throughout the left orbit that surrounds the globe and extends into the periorbital soft tissues (arrows)

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Fig. 3.13  A 14-year-old male with a “lump” in the left lateral superior orbital rim due to a mucoepidermoid carcinoma. (a) Axial T2 MRI with fat saturation shows a small hyperintense mass with a hypointense rim in an enlarged left lacrimal gland (arrow). (b, c) Axial and coro-

nal T1 post-contrast MRI with fat saturation shows peripheral enhancement of the small mass within the enlarged lacrimal gland (arrows)

Imaging

MRI

• Low-grade MEC may appear radiographically similar to a pleomorphic adenoma [33] while high-grade MEC may resemble an adenoid cystic carcinoma [1]. • Margins may be smooth or irregular [33, 36]. • Lower-grade MEC without mucinous components may also have irregular margins [38]. • Imaging is often not characteristic for definitive diagnosis, and biopsy may be required [33].

• T1 hypointense and T2 hypo- to hyperintense with heterogeneous enhancement. • Mucin-containing cystic components in low-grade MEC appear T1 and T2 hyperintense [33].

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Key Points

Imaging

• Low-grade MEC may appear similar to a pleomorphic adenoma, while high-grade MEC may resemble adenoid cystic carcinoma.

• May have benign [40, 41] or aggressive features [27, 30]. • Lobular lesion of the superotemporal orbit.

CT

Squamous Cell Carcinoma

• Bone erosion or destruction can be seen [40]. Figures 3.14 shows a case of lacrimal squamous cell carcinoma.

Background • Rare lacrimal gland neoplasm that may arise as a primary or secondary tumor. • Primary lesions may arise through squamous transformation of a pleomorphic adenoma or malignant degeneration of epithelial lined cysts, e.g., lacrimal duct cysts (dacryops) and choristomatous cysts (epidermoid and dermoid cysts). • Secondary etiologies also include posttraumatic and postsurgical implantation cysts and lacrimal gland metastases [39].

MRI • T1 isointense and T2 iso- to hyperintense heterogeneous mass. • Ill-defined margins with local infiltration of the extraconal fat [41].

Key Points • May have benign or aggressive features.

Presentation • Symptoms: paresthesia, pain, ptosis, diplopia, enlarging lacrimal mass [39].

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Fig. 3.14  An 80-year-old male with left orbital pain and decreased visual acuity with a blind spot. (a) Axial CT with contrast, soft tissue window shows a peripherally enhancing left lacrimal gland squamous cell carcinoma with involvement of the eyelids as well as the

c

periorbital and postseptal soft tissues (arrows). (b, c) Axial and coronal T1 post-contrast MRI with fat saturation shows a peripherally enhancing left lacrimal gland mass with involvement of the eyelids and periorbital and postseptal soft tissues (arrows)

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Lymphoma Figures 3.15 and 3.16 show cases of lacrimal gland lymphoma.

Background • Lymphoproliferative disorders comprise approximately 50% of non-epithelial lesions of the lacrimal gland [12]. • The majority are malignant lymphomas, most of which are of the non-Hodgkin B-cell type, specifically the mucosal-associated lymphoid tissue (MALT) subtype [42, 43].

Presentation

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• Classically presents as a painless superotemporal orbital mass. • May be associated with Sjögren syndrome. –– Approximately 35% of patients with Sjögren syndrome develop lymphoma [12].

Imaging • Unilateral or bilateral. • Involvement typically of both the palpebral and orbital lobes of the lacrimal gland [1]. • Tends to mold to the orbit and around the globe rather than infiltrating or indenting the globe [11, 44]. • Bone remodeling or sclerosis may be present. • Usually does not cause bone destruction [1, 11, 44].

• Majority of patients present in the seventh decade of life [42, 43]. a

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Fig. 3.15  A 50-year-old female with right lacrimal and parotid masses due to lymphoma. (a) Axial T2 MRI with fat saturation shows homogeneously isointense bilateral lacrimal gland lymphoma (arrows). (b, c) Axial and coronal T1 post-contrast MRI with fat saturation shows homogeneously enhancing bilateral lacrimal masses (arrows) that

are molding around the globes. (d) Axial 18F-FDG PET/CT shows FDG-avid bilateral lacrimal gland lymphoma (arrows). (e) Axial T2 MRI with fat saturation shows an enlarged hyperintense right intraparotid node (arrow). (f) Axial 18F-FDG PET/CT shows FDG avidity of the right intraparotid node (arrow)

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Fig. 3.16  A 58-year-old female with left eye swelling due to lymphoma. (a) Axial T2 MRI with fat saturation shows homogeneously isointense left lacrimal gland lymphoma (arrow). (b) Axial T1 post-­ contrast MRI with fat saturation shows a homogeneously enhancing

left lacrimal mass (arrow). (c) DWI shows a hyperintense signal of the left lacrimal gland mass (arrow). (d) Apparent diffusion coefficient map shows corresponding hypointense signal in keeping with restricted diffusion of the lacrimal gland lymphoma (arrow)

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CT

Background

• Homogeneous, well-defined, isodense mass that shows homogeneous enhancement [11, 44].

• A solitary fibrous tumor (SFT), formerly named hemangiopericytoma [48], is a spindle-cell neoplasm that originates from mesenchymal tissue and most frequently occurs in the pleura [49]. • Extrapleural sites include the chest, abdomen, pelvis, head and neck, and meninges [50, 51]. • May occur in the postseptal orbit, lacrimal gland, lacrimal sac, and on the lower eyelid [49, 50].

MRI • Homogeneous soft tissue mass that is T1 isointense and T2 iso- to slightly hyperintense to muscle and demonstrates homogeneous enhancement [1]. • Diffusion-weighted imaging (DWI) shows restricted diffusion with lower ADC values when compared to other lacrimal gland diseases [45].

PET • High 18F-FDG uptake is noted on PET studies except for low-grade MALT lymphoma, which may show relatively low FDG uptake [46]. • Whole-body PET/CT is used for staging of orbital lymphomas and detection of systemic metastases [47].

Presentation • A wide age range has been reported, from 9 to 76 years, without gender predilection [52, 53]. • Symptoms: slowly progressive unilateral exophthalmos or palpable mass [54].

Imaging • Well-defined, ovoid mass.

Key Points

CT

• Non-destructive mass that molds to the orbit, including the globe. • Search for secondary sites of disease. • Search for nodes in the head and neck.

• SFTs are isodense to slightly hyperdense to white matter on non-contrast CT [55, 56]. • Rapid enhancement following contrast administration [55].

Solitary Fibrous Tumor

• T1 homogeneously isointense [56]. • T1 hypointense components are related to cystic or myxoid degeneration [57].

Figure 3.17 shows a case of a lacrimal solitary fibrous tumor.

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Fig. 3.17  A 38-year-old female with fullness in the right upper eyelid from a solitary fibrous tumor. (a) Axial T2 MRI with fat saturation shows a heterogeneous isodense right lacrimal gland solitary fibrous tumor (arrow) with a hyperintense cystic component (thin arrow). (b)

Axial T2 MRI with fat saturation shows punctate and linear vascular flow voids (arrow). (c) Axial T1 post-contrast MRI with fat saturation shows homogeneous enhancement of the solid component (arrow) without enhancement of the cystic component (thin arrow)

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• T2 iso- to hypointense related to fibrous tissue with a high collagen content. • Internal hemorrhage, cystic degeneration, or fresh fibrosis are T2 hyperintense [55]. • Signal flow voids from vessels may be noted on T2 images [56]. • Rapid enhancement with a washout pattern of contrast may aid in diagnosis [58]. • Slight restricted diffusion has been described [54].

Key Points • T2 iso- to hypointense with rapid uptake of contrast; signal flow voids may be present.

Idiopathic Orbital Inflammation Figure 3.18 shows a case of idiopathic orbital inflammation with involvement of the lacrimal gland.

Background • Idiopathic orbital inflammation (IOI) is an inflammatory condition that was previously referred to as an orbital pseudotumor. • Histologically characterized by the infiltration of polymorphous lymphocytes and a variable degree of fibrosis [59, 60].

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Fig. 3.18  A 58-year-old male with diplopia and swelling around the left eye from idiopathic orbital inflammation. (a) Axial T2 MRI without fat saturation shows homogenously isointense disease involving the left lacrimal gland, eyelid and retrobulbar soft tissues (arrows). (b,

• Following thyroid orbitopathy and lymphoproliferative disorders, IOI is the third most common disease to affect the orbit [61].

Presentation • Symptoms: headache, periorbital pain, and inflammatory signs such as erythema and swelling. • Lacrimal gland involvement can cause dacryoadenitis. • Compression upon the orbital apex and cavernous sinus involvement may lead to cranial nerve palsies and decreased visual acuity [62].

Imaging • Lacrimal gland enlargement. • Nonspecific inflammatory soft tissue in the orbit with infiltration of the orbital fat. • If the extraocular muscles are involved, IOI may include the tendinous portion of the muscles. • Other sites of involvement include the optic nerve, including the junction with the globe and adjacent periorbital soft tissues. • Retro-orbital involvement may occur from extension through the superior and inferior orbital fissures and through the optic canal to the cavernous sinus [59].

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c) Axial and coronal T1 post-contrast MRI with fat saturation shows homogeneously enhancing disease in the superotemporal left orbit and periorbital soft tissues (arrow). Note inferior displacement of the left globe (thin arrow)

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CT

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• Search for other sites of disease in the orbit, cavernous sinus, and cranial nerves. • May include the tendinous portion of the extraocular muscles as opposed to Graves’ eye disease.

• Enhancement with contrast [62].

MRI • T1 isointense and T2 iso- to hypointense may be due to fibrosis. • Variable contrast enhancement [62, 63]. • Restricted diffusion has been described [64].

Immunoglobulin G4-Related Disease

Key Points

Background

• IOI can be treated with corticosteroids, usually resulting in rapid decrease in the size of the mass for most patients. Recurrence is possible.

• Immunoglobulin G4-related disease (IgG4-RD) is a systemic disease of unknown etiology.

Figures 3.19 and 3.20 show cases of Immunoglobulin G4-related disease.

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Fig. 3.19  A 37-year-old female with chronic enlargement of the lacrimal glands due to IgG4-RD. (a, b) Axial and coronal T1 post-contrast MRI with fat saturation shows homogeneously enhancing masses involving bilateral lacrimal glands and retrobulbar soft tissue

(arrows). (c) Axial T1 post-contrast MRI with fat saturation shows enhancing soft tissue in the right and possibly left pterygopalatine fossae (arrows). (d) Axial 18F-FDG PET/CT shows FDG-avid disease in the right pterygopalatine fossa only (arrow)

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Fig. 3.20  A 43-year-old male with right lacrimal swelling due to IgG4-RD. (a) Axial T1 post-contrast MRI with fat saturation shows a homogeneously enhancing lesion in the right lacrimal gland and intraconal space (arrows). (b) Coronal T1 post-contrast MRI with fat saturation shows enhancing disease surrounding the right optic

nerve (white arrow) with asymmetric extraocular muscle enlargement (black arrow) and involvement of bilateral infraorbital nerves (V2) (thin arrows). (c) Axial T1 post-contrast MRI with fat saturation shows enhancing disease involving bilateral infraorbital nerves (V2) (arrows) and the right cavernous sinus (thin arrow)

• Characterized by inflammation, fibrosis, and tissue infiltration with plasma cells that express IgG4. • Various organs may be involved, including the pancreas, bile duct, liver, retroperitoneal soft tissues, lung, thyroid, salivary glands, and lymph nodes, either alone or systematically [65, 66]. • After the pancreas, the head and neck region is the second most affected site [67].

• Other possible findings about the orbits include enlargement of the extraocular muscles, infiltration of the orbital fat, cranial nerve involvement, especially the infraorbital nerve (V2), soft tissue in the cavernous sinuses, and Meckel’s cave [72]. • Associated paranasal sinus inflammatory mucosal thickening has been reported [72].

Presentation • Related symptoms: hypophysitis, thyroiditis, pancreatitis, cholecystitis, retroperitoneal fibrosis, and lymphadenopathy [68–71]. • Predominantly in older men, often with elevated serum IgG4 levels [68, 72]. • Lacrimal involvement (dacryoadenitis) may occur in isolation or with salivary gland involvement (sialadenitis) as part of Mikulicz’s disease (painless bilateral enlargement of the lacrimal, parotid, and submandibular glands) [70]. • Other sites of disease in the head and neck include the orbit, pituitary gland, cavernous sinus, cranial nerves, paranasal sinuses, and cervical nodes [67].

CT • Homogeneous soft tissue density of the disease with contrast enhancement.

MRI • T1 hypointensity, T2 hypo- to hyperintensity with homogeneous enhancement [68, 71, 72].

PET • IgG4-RD cases are 18F-FDG-avid, and PET is useful to detect multi-organ involvement, guide biopsies, and assess treatment response [68, 73].

Imaging

Key Points

• The most common sites are the lacrimal gland and extraocular muscles, which are affected in the majority of patients with this diagnosis [72]. • Lacrimal glands are enlarged, which may be unilateral or bilateral.

• Search for other sites of disease in the orbit, cavernous sinuses, and cranial nerves. • Search for other sites of disease involvement throughout the body.

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Sarcoidosis

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Figure 3.21 shows a case of sarcoidosis involving the lacrimal gland.

• The cranial nerves, leptomeninges, brain parenchyma, and hypothalamic-pituitary axis may also be involved. Diabetes insipidus may result [77]. • The parotid gland is involved in 5% of cases [78].

Background

Imaging

• Chronic systemic multiorgan disorder characterized by a non-caseating granulomatous reaction. • Unknown etiology. • Can affect nearly every organ in the body, with orbital involvement reported in 25–83% of patients [74, 75]. • The lungs, skin, orbit, and lymph nodes are most commonly affected. • The liver, kidneys, heart, and brain may also be involved [74, 75]. • Biopsy is often required and demonstrates non-­ caseating granulomas [76].

• In addition to the lacrimal gland, sarcoidosis can involve the orbital fat, extraocular muscles, and optic nerve sheath [82]. • Displacement of the globe including proptosis may be present [83].

Presentation • Primarily develops in patients aged 25–45  years old, although children and older individuals may also be affected [77]. • Ocular involvement in 20% of patients [78]. • African Americans have a higher incidence of ocular involvement compared to Whites [79]. • Anterior uveitis followed by posterior uveitis can severely impact visual acuity [80]. • Other findings: enlarged hilar lymph nodes and pulmonary infiltrates. • High serum angiotensin-converting enzyme level is present but not specific [81].

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CT • Diffuse lacrimal gland enlargement with homogeneous enhancement [82].

MRI • Diffusely enlarged and enhancing lacrimal glands. • Extraocular muscle involvement characterized by abnormal thickening and enhancement of the muscles and tendinous insertions. • Infiltrative and enhancing soft tissue can be seen in the retrobulbar fat [78]. • The optic nerves may show thickened enhancing nodules with T2 signal hyperintensity of the nerve [84].

Nuclear Medicine •

F-FDG PET/CT and Gallium scans aid in diagnosis when orbital, parotid, and bilateral hilar uptake is detected [85]. 18

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Fig. 3.21  A 51-year-old female with sarcoidosis and bilateral lacrimal gland enlargement. (a) Axial CT with contrast, soft tissue window shows enlargement of bilateral lacrimal glands (arrows). (b) Plain

radiograph shows bilateral hilar enlargement (arrows). (c) Coronal CT with contrast, soft tissue window shows paratracheal and bilateral hilar adenopathy (arrows)

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Key Points

Background

• Search for other sites of involvement throughout the body (e.g., regional lymph nodes).

• Autoimmune disease with lymphocytic infiltration and destruction of the exocrine glands [86, 87]. • The salivary and lacrimal glands are targeted, producing the sicca syndrome of dry mouth and dry eyes. • Diagnosis may require biopsy of the lacrimal gland with rose Bengal staining [1].

Sjögren’s Syndrome Figure 3.22 shows cases of Sjögren’s syndrome involving the lacrimal glands.

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Fig. 3.22 (a, b) A 41-year-old female with Sjögren’s syndrome. (a) Axial T1 post-contrast MRI with fat saturation shows an atrophic appearance of bilateral lacrimal glands (arrows). (b) Coronal T1 post-contrast MRI with fat saturation shows an enlarged cystic appearance of bilateral parotid and submandibular glands (arrows) with an enlarged right neck node (thin arrow). (c, d) A 45-year-old female

with dry mouth and eyes and a right lacrimal mass diagnosed as lymphoma. (c) Axial T1 post-contrast MRI with fat saturation shows right lacrimal gland lymphoma (arrow). (d) Axial T2 MRI with fat saturation shows a cystic appearance of bilateral parotid glands, suggesting Sjögren’s syndrome (arrows)

3  Lacrimal Gland and Nasolacrimal Drainage Apparatus

• Two forms exist, primary and secondary, the latter of which is associated with other autoimmune diseases, mostly rheumatoid arthritis [87]. • With Sjögren’s syndrome, the relative risk of developing lymphoma is approximately 16–18 times greater than normal [88, 89].

Presentation • Tends to occur in women over 40 years of age but can occur in children [87, 90]. • Gland involvement is usually bilateral and diffuse [1]. • Dryness of the eye can be complicated by dryness of the mouth, keratoconjunctivitis, blepharitis (eyelid inflammation), and corneal inflammation and ulceration [91]. • Rare systemic manifestations include uveitis, episcleritis, and idiopathic orbital inflammation [91]. • Markers: anti-Ro (SSA) antibody and anti-La (SSB) antibody [92].

Imaging

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MRI • Patchy regions of T1 signal hyperintensity related to fatty infiltration with subtle heterogeneous enhancement [1, 93].

Key Points • Usually bilateral lacrimal gland enlargement. • Enlargement of lacrimal glands in early stage and atrophy in late stages. • Greater risk of developing lymphoma.

Xanthogranuloma Figure 3.23 shows a case of xanthogranuloma involving the lacrimal gland.

Background

• In the early stage, the lacrimal glands may be normal or enlarged in size. • In the later stages, the glands are atrophic with a reticular or multicystic pattern [93].

• Xanthogranuloma is a non-Langerhans cell histiocytosis consisting of infiltrates of histiocytes, often with abundant intracellular lipids, multinucleate Touton giant cells, and inflammatory cells. • Occurs at birth (20%), in the first year of life (70%), and in adulthood (10%) [94].

CT

Presentation

• Bilateral lacrimal gland enlargement or atrophy depending on the stage. • No bone involvement [1].

• Can be cutaneous or systemic affecting multiple organs, including the liver, spleen, kidneys, lungs, eyes, bones, and central nervous system [95–97].

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Fig. 3.23  A 55-year-old male who presented with a left orbital mass from xanthogranuloma. (a) Axial T1 non-contrast MRI without fat saturation shows homogeneous isointense masses involving bilateral lacrimal glands (arrows). (b) Axial T2 MRI with fat saturation shows

a hypointense appearance of the xanthogranuloma (arrows). (c) Axial T1 post-contrast MRI with fat saturation shows homogeneous enhancement of the masses (arrows)

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• Cutaneous xanthogranuloma often presents as a solitary nodule that is flesh-colored, erythematous, brown, or yellow in appearance due to a high lipid component [95–98]. • Common sites in the head and neck include the skin of the eyelid, the midline nasofrontal and periauricular soft tissues, and the temporal bone. • Intracranial disease may present as a dural-based mass [94, 99].

• Search for other sites of disease involvement throughout the body.

Imaging

• Herniation of the lacrimal gland outside of the lacrimal fossa. • Etiologies: congenital, acquired relaxation of local suspending ligaments related to aging and trauma [101].

• Bone erosion is uncommon. • When bone involvement is present, xanthogranuloma cannot be radiologically distinguished from Langerhans cell histiocytosis [99].

Lacrimal Gland Prolapse Figure 3.24 shows cases of lacrimal gland prolapse.

Background

Presentation

CT

• Bulge in the lateral third of the upper eyelids.

• Homogeneously hypodense to muscle. • Homogeneous enhancement in a well-circumscribed nodule or mass [94].

Imaging

MRI • T1 iso- to hyperintense, T2 hypo- to isointense possibly related to the presence of lipid. • Demonstrates homogeneous enhancement. • Restricted diffusion may be present on DWI sequences due to a collagenous matrix [94, 100].

Key Points • Biopsy and immunochemistry may be required to differentiate from other histiocytoses.

• Palpebral lobe of the lacrimal gland located outside the lacrimal fossa anterior, inferior and lateral to the orbital rim. • No discrete mass in the lacrimal gland.

Key Points • Similar appearance to normal lacrimal tissue without a defined mass.

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Fig. 3.24 (a–c) A 40-year-old female with a prolapsed right lacrimal gland. (a, b) Axial T1 post-contrast MRI with fat saturation shows prolapse of the right lacrimal gland anterior to the orbital rim with a similar appearance as normal lacrimal tissue (arrows). (c) Coronal T1 post-contrast MRI with fat saturation shows anterior and inferior

prolapse of the right lacrimal gland (arrow). (d) A 54-year-old female with left blurry vision. (d) Axial CT with contrast, soft tissue window shows prolapse of bilateral lacrimal glands anterior to the orbital rims (arrows)

Benign Lymphoid Hyperplasia

• A monoclonal B-cell population excluded with flow cytometry and molecular techniques. • May undergo malignant transformation to B-cell lymphoma or lymphoepithelial carcinoma [103].

Figure 3.25 shows cases of benign lymphoid hyperplasia.

Background • Lymphoproliferative disorder with infiltration of tissue predominantly by polymorphic B-lymphocytes [102].

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Presentation • Can involve the lacrimal gland, nasolacrimal duct, or conjunctiva [104]. • Slow-growing, firm, mobile mass most often occurring in the fourth to fifth decade.

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• Presentation may be similar to lymphoma [102]. • Bilateral disease: 15–36%. • Presenting signs: lacrimal gland enlargement, proptosis, and orbital or eyelid swelling [102]. • Pain and discomfort are less common features [104].

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Fig. 3.25 (a, b) A 79-year-old female with a drooping right eyelid and right lacrimal gland hyperplasia. (a, b) Axial and coronal CT with contrast, soft tissue window shows homogeneous enlargement of the right lacrimal gland (arrows). (c, d) A 39-year-old female with epiphora (excessive tearing) and bilateral lacrimal gland hyperplasia. (c)

Axial T2 MRI with fat saturation shows a homogeneously isointense appearance of enlarged lacrimal glands (arrows). (d) Axial T1 post-­ contrast MRI shows homogeneous enhancement of prominent lacrimal glands (arrows)

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Imaging • Lesions mold to the orbital walls and around the globe and optic nerve often with minimal displacement of the globe. • Rounded or lobulated with diffuse enhancement. • Cortical bone is intact without invasion. • Radiologically, lymphoid hyperplasia may indistinguishable from low-grade non-Hodgkin’s lymphoma on CT and MRI. Biopsy may be required [104].

CT • Isodense and homogeneous with diffuse enhancement [104].

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• Inflammation around the lacrimal gland ducts and trauma with duct obstruction cause lacrimal gland hypersecretions and passive dilation of the ducts with subsequent cyst formation [105].

Presentation • Painless swelling along the lateral aspect of the upper eyelid. • Hemorrhage and superinfection can occur. • Upper eyelid eversion reveals a fluid-filled lesion.

Imaging • Lacrimal gland cysts are often surrounded by glandular tissue and dilated lacrimal ducts [106].

MRI • T2 hypo- to isointense due to dense packing of lymphocytes with diffuse enhancement [104].

CT • Fluid density lacrimal gland cysts without evidence of bony abnormalities [107, 108].

Key Points • May indistinguishable from low-grade non-Hodgkin’s lymphoma.

MRI • Well-defined small cysts are T1 hypointense and T2 hyperintense [109]. • Cysts are best visualized on T1 post-contrast imaging with fat suppression and T2 MRI sequences.

Dacryops Figure 3.26 shows a case of dacryops.

Background

Dermolipoma

• Dacryops (lacrimal cysts) occur from obstruction of the lacrimal gland ducts, leading to cyst formation.

Figure 3.27 shows a case of a dermolipoma.

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Fig. 3.26  A 44-year-old female with a painless bump on the left eyelid. (a) Axial T2 MRI with fat saturation shows homogeneously hyperintense cysts in the left lacrimal gland (arrow). (b, c) Axial and coronal

T1 post-contrast MRI with fat saturation shows a homogeneous non-­ enhancing left lacrimal cyst (arrows)

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Fig. 3.27  A 54-year-old male with extranodal marginal zone lymphoma and a right superotemporal dermolipoma. (a) Axial T1 non-­ contrast MRI without fat saturation shows a homogeneously hyperintense dermolipoma (arrow) anterior to the right lacrimal

fossa. The dermolipoma is not continuous with the intraconal fat and lies along the lateral wall of the globe. (b) Axial T2 MRI with fat saturation shows a hypointense appearance of the dermolipoma due to fat saturation (arrow)

Background

Presentation

• Dermolipoma is a congenital mass containing adipose tissue and dermal-like connective tissue [110, 111]. • Probably arises from sequestration during embryonic development of the eyelid [111].

• Symptoms: orbital pain sometimes localized to the lacrimal fossa, edema, and erythema of the lateral upper eyelid, proptosis, pain and limitation of eye movement, and diplopia. • The lacrimal gland may be palpable, sometimes visualized with eyelid eversion. • Injection of the conjunctiva is common. • An enlarged gland can cause the globe to be displaced in an inferonasal direction [113, 114]. • Can occur following an acute infection or related to conditions such as sarcoidosis, Mikulicz’s syndrome, thyroid eye disease, tuberculosis, and granulomatosis with polyangiitis (Wegener’s granulomatosis) [115].

Presentation • Unilateral fatty mass in the lateral canthus [112].

Imaging • Homogeneous crescent or triangular fatty mass in the temporal or superotemporal epibulbar region. • Located medial to the lacrimal gland, anterior to the lateral rectus muscle insertion, and adjacent to the lateral wall of the globe. • No connection with intraconal fat. • Subtle soft tissue stranding within the mass. • No enhancement or calcification [112].

Dacryoadenitis Figure 3.28 shows cases of dacryoadenitis.

Background • Lacrimal gland inflammation can be infectious or noninfectious and may occur unilaterally or bilaterally [113, 114].

Imaging • Acute dacryoadenitis presents with marked enhancement within the lacrimal gland and surrounding soft tissues with no bony involvement [1]. • Enlargement of the extraocular muscles. • Thickening or stranding of the periorbital or retrobulbar fat. • Associated scleritis may present with uveoscleral enhancement and fluid in Tenon’s space [116]. • Chronic dacryoadenitis presents with enlargement of the lacrimal gland. –– Increased thickness of the lacrimal bone [115].

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Fig. 3.28 (a) A 66-year-old male with myelodysplastic syndrome and left eye swelling due to dacryocystitis. (a) Axial CT with contrast, soft tissue window shows enlargement of the left lacrimal gland and soft tissue in the left upper eyelid (arrows) diagnosed clinically as dacryocystitis. (b, c) A 73-year-old female with breast cancer and right conjunctivitis. (b) Axial CT with contrast, soft tissue window shows

enlargement of the right lacrimal gland and soft tissue in the right upper eyelid (arrows). (c) Coronal CT with contrast, soft tissue window shows enlargement of the right lacrimal gland and superior rectus muscle complex (arrows) with soft tissue stranding in the intraconal space (thin arrow) diagnosed clinically as dacryocystitis

• The nasolacrimal duct may be dilated or eroded [117]. • Intracranial spread or nodal metastases to the pre-­ auricular, submandibular, and neck nodes may be present but occurs less often [120]. • Perineural spread and distant metastases are rare [117].

Lacrimal Sac Lesions Squamous Cell Carcinoma Figures 3.29, 3.30, and 3.31 show cases of lacrimal sac squamous cell carcinoma.

Background • Squamous cell carcinomas can have varying degrees of differentiation from well-differentiated to poorly differentiated [7].

Presentation • Generally present in the adult population [117, 118]. • When presenting early in the disease course, the symptoms may appear as a benign process such as dacryocystitis or nasolacrimal duct obstruction [118]. • Other signs and symptoms: mass in the lacrimal sac, epiphora (excessive tearing), blood-stained tears, and epistaxis [119]. • Exophthalmos and diplopia may occur when the tumor extends directly into the orbit.

Imaging • A mass may be visualized in the lacrimal sac or nasolacrimal duct.

CT • The tumors will show enhancement with contrast [117]. • CT can be used to determine the presence of bone erosion [119].

MRI • T1 isointense and T2 hypo- to isointense and enhance with contrast [117, 121]. –– Heterogeneous enhancement with tumor necrosis. • MRI can be used to determine the extent of tumor spread and to separate tumor from inflammatory sinonasal mucosa, secretions, and adipose tissues [119].

PET/CT •

F-FDG-avid on PET/CT [122].

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Key Points • Mention if there is bone destruction and any extension of tumor into the orbit, paranasal sinuses, and/or premaxillary soft tissues.

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Fig. 3.29  A 75-year-old male with a left medial canthus swelling biopsied as squamous cell carcinoma. (a) Axial T1 post-contrast MRI with fat saturation shows a complex partially solid and necrotic mass in the left lacrimal sac and medial canthus (arrow) (b) Coronal T1 post-­ contrast MRI with fat saturation shows a left lacrimal sac mass with

extension into the nasolacrimal duct (arrows). (c) Axial 18F-FDG PET/CT shows an FDG-avid mass in the left lacrimal sac and medial canthus (arrow). (d) Axial 18F-FDG PET/CT shows an FDG-avid mass in the left nasolacrimal duct (arrow)

3  Lacrimal Gland and Nasolacrimal Drainage Apparatus Fig. 3.30  A 63-year-old male with left epiphora (excessive tearing) and left medial canthus swelling with a mass biopsied as basaloid squamous cell carcinoma. (a) Axial CT with contrast, soft tissue window shows a large homogeneously enhancing mass in the left lacrimal sac and medial postseptal space. There is destruction of left nasal bone and lamina papyracea with tumor extending into the left sinonasal cavity (arrows) (b) Coronal CT with contrast, soft tissue window shows tumor extending into the left nasal cavity (arrow). (c) Axial 18F-FDG PET/CT shows an FDG-avid left submandibular node. (d) Ultrasound, transverse and longitudinal planes shows a metastatic left submandibular node (arrows)

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Fig. 3.31  A 51-year-old male with pain and blurry vision with a right facial mass biopsied as poorly differentiated squamous cell carcinoma. (a) Axial T2 MRI with fat saturation shows an isointense mass in the right premaxillary soft tissues with involvement of the right naso-

lacrimal duct and sinonasal cavity (arrows). (b) Coronal T1 post-­ contrast MRI with fat saturation shows an enhancing lacrimal sac and medial canthus mass extending into the inferonasal right orbit and right maxillary sinus (arrows)

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Lymphoma

• Some cases are misdiagnosed as dacryocystitis [125, 128].

Figure 3.32 shows a case of lacrimal sac lymphoma.

Background

Imaging • Lacrimal sac mass without or with bone destruction [125].

• Malignant lymphoma accounts for approximately 6% of lacrimal sac tumors [123]. • The majority of lacrimal sac lymphomas are MALT lymphoma, and diffuse large B-cell lymphoma [124].

Presentation

CT • Iso- to slightly hyperdense and may appear cystic [129].

MRI

• Mainly occurs in the older population [125]. • Reported to arise in women more often than men [126]. • Symptoms: nonspecific and include dacryocystitis, epiphora, pain, swelling, and blood in tears [127].

• T1 isointense and T2 hypo- to isointense [130]. • Lesions are generally homogeneous and demonstrate homogeneous enhancement [129].

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f

Fig. 3.32  A 57-year-old female with left epiphora (excessive tearing) and a left lacrimal sac/medial canthus follicular lymphoma. (a) Axial T2 MRI with fat saturation shows an isointense appearance of the lacrimal sac and medial canthus mass (arrow). (b) Coronal T1 post-­ contrast MRI with fat saturation shows extension of the homogeneously enhancing mass into the proximal nasolacrimal duct

(arrow). (c) DWI shows hyperintense signal in the lacrimal sac/medial canthus lymphoma (arrow). (d) Apparent diffusion coefficient map shows corresponding hypointense signal of the mass consistent with restricted diffusion of the lymphoma (arrow). (e, f) Coronal 18F-FDG PET/CT shows an FDG-avid mass with involvement of the NLDA (arrows)

3  Lacrimal Gland and Nasolacrimal Drainage Apparatus

PET/CT • High 18F-FDG uptake is noted on PET studies except for low-grade MALT lymphoma which may show relatively low 18F-FDG uptake [46]. • Whole-body PET/CT is used for staging and detecting systemic metastases [47].

Key Points • If a painless mass is found in the lacrimal sac, lymphoma should be differentiated from carcinoma, pseudotumor, and an inflammatory lesion.

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• Clinically, symptoms may resemble dacryocystitis and may lead to misdiagnosis. • The most frequent symptoms are bloody discharge and swelling [123].

Imaging • Soft tissue mass in the lacrimal fossa that can initially involve the nasolacrimal duct without bone destruction. • Over time, they invade adjacent bone and soft tissue, including the orbit and paranasal sinuses [131]. • Cysts are uncommon. • Hemorrhage within the lesion may occur [132].

Melanoma

CT

Figures 3.33 and 3.34 show cases of lacrimal sac melanoma.

• Isodense to slightly hyperdense mass calcification. • CT can evaluate for bone destruction [132].

Background

MRI

• Lacrimal sac melanoma is a rare condition that is highly malignant. • Lacrimal sac melanoma is believed to develop from melanin cells that are left in the epithelium during embryonic development. • Can metastasize at an early stage. • Patients have a poor prognosis [7].

Presentation

• Lacrimal sac melanoma may contain fewer melanocytes than melanoma at other locations. • Thus, melanoma in the lacrimal sac can be T1 isointense and T2 iso- to hyperintense and enhance [132].

PET/CT • Lacrimal sac melanoma has been reported to be 18F-FD-­ avid on PET/CT [133].

• The average age at presentation is 59 years (range, 27–81 years) with no gender bias.

Fig. 3.33  An 88-year-old with right epiphora (excessive tearing) and a right lacrimal sac melanoma. (a, b) Axial and coronal CT with contrast, soft tissue window shows a heterogeneously enhancing melanoma in the right lacrimal sac with destruction of right nasal bone and lamina papyracea with extension into the right nasal cavity (arrows)

without

a

b

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a

J. M. Debnam et al.

b

c

Fig. 3.34  A 55-year-old male with right epiphora (excessive tearing) and a right lacrimal sac melanoma. (a) Axial T1 non-contrast MRI without fat saturation shows a hyperintense appearance of the melanoma that may be related to melanin and/or hemorrhage (arrow).

(b) Axial T2 MRI with fat saturation shows an iso- to hypointense appearance of the melanoma (arrow). (c) Axial T1 post-contrast MRI with fat saturation shows homogeneous enhancement of the melanoma (arrow)

Key Points

Dacryocystitis

• Symptoms: epiphora, pain, and mucopurulent discharge. • Secondary symptoms: conjunctivitis, periorbital cellulitis, and abscess [134, 135]. • Most cases are diagnosed on clinical examination and do not require imaging. • Imaging is indicated to exclude postseptal involvement, which is rare because the orbital septum acts as a barrier against postseptal spread of the infection [136].

Figure 3.35 shows cases of dacryocystitis.

Imaging

Background

• Imaging is indicated to rule out secondary orbital cellulitis, orbital abscesses, foreign bodies, and tumors [137]. • Lacrimal sac infection appears as a thickened, well-­ circumscribed rounded lacrimal sac with peripheral enhancement [135]. • Medial canthus and periorbital swelling [138].

• Mention if there is bone destruction and any extension of tumor into the orbit, paranasal sinuses, and/or premaxillary soft tissues.

• Inflammation of the NLDA caused by impairment in the drainage system and superimposed infection. • Obstruction of the NLDA usually is the causative factor [134]. • The obstruction may be congenital or acquired. • Acquired etiologies include inflammation/infection, dacryolithiasis, trauma, and tumors [135].

Presentation • Bimodal age distribution predominantly affecting neonates and adults over 40 years [47] [134].

Key Points • Important not to confuse infection of the lacrimal sac with an abscess. • Search for a causative tumor or foreign body.

3  Lacrimal Gland and Nasolacrimal Drainage Apparatus

113

a

b

c

d

Fig. 3.35 (a, b) A 23-year-old female with acute lymphoid leukemia and left periorbital swelling. (a) Axial CT with contrast, soft tissue window shows thickened enhancement of the left lacrimal sac and soft tissue thickening of the left upper eyelid (arrows). (b) Coronal CT with contrast, soft tissue window shows thickened enhancement of the left lacrimal sac with adjacent soft tissue stranding (arrow) consistent with dacryocystitis. (c, d) A 76-year-old female with acute

myeloid leukemia who presented with right conjunctivitis with periorbital swelling. (c) Axial T1 post-contrast MRI with fat saturation shows an abscess anterior to the right lacrimal sac with a fistulous communication to the skin surface (arrow). (d) Coronal T1 post-contrast MRI with fat saturation shows an abscess anterior to the right lacrimal sac (arrow) with adjacent soft tissue thickening and enhancement (thin arrows)

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J. M. Debnam et al. characteristics of lymphoma development in primary Sjögren’s syndrome. Semin Arthritis Rheum. 2011;41:415–23. https://doi. org/10.1016/j.semarthrit.2011.04.006. 90. Gomes Pde S, Juodzbalys G, Fernandes MH, Guobis Z. Diagnostic approaches to Sjögren’s syndrome: a literature review and own clinical experience. J Oral Maxillofac Res. 2012;3:e3. https://doi. org/10.5037/jomr.2012.3103. 91. Parisis D, Chivasso C, Perret J, Soyfoo MS, Delporte C. Current state of knowledge on primary Sjögren’s syndrome, an autoimmune exocrinopathy. J Clin Med. 2020;9:2299. https://doi. org/10.3390/jcm9072299. 92. Shiboski CH, Shiboski SC, Seror R, Criswell LA, Labetoulle M, Lietman TM, et  al. 2016 American College of Rheumatology/ European League Against Rheumatism classification criteria for primary Sjögren’s syndrome: a consensus and data-driven methodology involving three international patient cohorts. Ann Rheum Dis. 2017;76:9–16. https://doi.org/10.1136/annrheumdis-­2016-­210571. 93. Izumi M, Eguchi K, Uetani M, Nakamura H, Takagi Y, Hayashi K, et al. MR features of the lacrimal gland in Sjögren’s syndrome. AJR Am J Roentgenol. 1998;170:1661–6. https://doi.org/10.2214/ ajr.170.6.9609194. 94. Ginat DT, Vargas SO, Silvera VM, Volk MS, Degar BA, Robson CD.  Imaging features of juvenile xanthogranuloma of the pediatric head and neck. AJNR Am J Neuroradiol. 2016;37:910–6. https://doi.org/10.3174/ajnr.A4644. 95. Janssen D, Harms D. Juvenile xanthogranuloma in childhood and adolescence: a clinicopathologic study of 129 patients from the kiel pediatric tumor registry. Am J Surg Pathol. 2005;29:21–8. https://doi.org/10.1097/01.pas.0000147395.01229.06. 96. Dehner LP.  Juvenile xanthogranulomas in the first two decades of life: a clinicopathologic study of 174 cases with cutaneous and extracutaneous manifestations. Am J Surg Pathol. 2003;27:579– 93. https://doi.org/10.1097/00000478-­200305000-­00003. 97. Freyer DR, Kennedy R, Bostrom BC, Kohut G, Dehner LP.  Juvenile xanthogranuloma: forms of systemic disease and their clinical implications. J Pediatr. 1996;129:227–37. https://doi. org/10.1016/s0022-­3476(96)70247-­0. 98. Tahan SR, Pastel-Levy C, Bhan AK, Mihm MC Jr. Juvenile xanthogranuloma. Clinical and pathologic characterization. Arch Pathol Lab Med. 1989;113:1057–61. 99. Hidayat AA, Mafee MF, Laver NV, Noujaim S.  Langerhans’ cell histiocytosis and juvenile xanthogranuloma of the orbit. Clinicopathologic, CT, and MR imaging features. Radiol Clin N Am. 1998;36:1229–40, xii. https://doi.org/10.1016/ s0033-­8389(05)70242-­6. 100. David JK, Anupindi SA, Deshpande V, Jaramillo D. Intramuscular juvenile xanthogranuloma: sonographic and MR findings. Pediatr Radiol. 2003;33:203–6. https://doi.org/10.1007/ s00247-­002-­0813-­5. 101. Friedhofer H, Orel M, Saito FL, Alves HR, Ferreira MC. Lacrimal gland prolapse: management during aesthetic blepharoplasty: review of the literature and case reports. Aesthet Plast Surg. 2009;3:647–53. https://doi.org/10.1007/s00266-­008-­9222-­y. 102. Ho HH, Savar A, Samaniego F, Manning J, Kasyan A, Pro B, et al. Treatment of benign lymphoid hyperplasia of the orbit with rituximab. Ophthalmic Plast Reconstr Surg. 2010;26:11–3. 103. Chen A, Hwang TN, Phan LT, McCulley TJ, Yoon MK. Long-term management of orbital and systemic reactive lymphoid hyperplasia with rituximab. Middle East Afr J Ophthalmol. 2012;19:432– 5. https://doi.org/10.4103/0974-­9233.102770. 104. Andrew NH, Coupland SE, Pirbhai A, Selva D. Lymphoid hyperplasia of the orbit and ocular adnexa: a clinical pathologic review. Surv Ophthalmol. 2016;61:778–90. https://doi.org/10.1016/j. survophthal.2016.04.004. 105. Duke-Elder S, MacFaul PA. Systems of ophthalmology series: the ocular adnexa, vol. 13. St. Louis, MO: Mosby; 1974. p. 638–42.

3  Lacrimal Gland and Nasolacrimal Drainage Apparatus 106. Smith S, Rootman J.  Lacrimal ductal cysts. Presentation and management. Surv Ophthalmol. 1986;30:245–50. https://doi. org/10.1016/0039-­6257(86)90120-­7. 107. Bradey N, Hayward JM.  Case report: bilateral lacrimal gland enlargement: an unusual manifestation of dacryops. Clin Radiol. 1991;43:280–1. https://doi.org/10.1016/s0009-­9260(05)80259-­4. 108. Khoury NJ, Haddad MC, Tawil AN, Ma’luf RN.  Ductal cysts of the accessory lacrimal glands: CT findings. AJNR Am J Neuroradiol. 1999;20:1140–2. 109. Zhang Y, Zeng C, Chen N, Liu C.  Lacrimal ductal cyst of the medial orbit: a case report. BMC Ophthalmol. 2020;20:380. https://doi.org/10.1186/s12886-­020-­01636-­1. 110. McNab AA, Wright JE, Caswell AG.  Clinical features and surgical management of dermolipomas. Aust N Z J Ophthalmol. 1990;18:159–62. https://doi.org/10.1111/j.1442-­9071.1990. tb00608.x. 111. Eijpe AA, Koornneef L, Bras J, Verbeeten B Jr, Peeters FL, Zonneveld FW.  Dermolipoma: characteristic CT appearance. Doc Ophthalmol. 1990;74:321–8. https://doi.org/10.1007/ BF00145816. 112. Kim E, Kim HJ, Kim YD, Woo KI, Lee H, Kim ST. Subconjunctival fat prolapse and dermolipoma of the orbit: differentiation on CT and MR imaging. AJNR Am J Neuroradiol. 2011;32:465–7. https://doi.org/10.3174/ajnr.A2313. 113. Andrew NH, Kearney D, Sladden N, McKelvie P, Wu A, Sun MT, et al. Idiopathic dacryoadenitis: clinical features, histopathology, and treatment outcomes. Am J Ophthalmol. 2016;163:148–153. e1. https://doi.org/10.1016/j.ajo.2015.11.032. 114. Luemsamran P, Rootman J, White VA, Nassiri N, Heran MKS. The role of biopsy in lacrimal gland inflammation: a clinicopathologic study. Orbit. 2017;36:411–8. https://doi.org/10.1080/01676830.2 017.1352608. 115. Bulgurcu S, Idil M, Pekçevik Y, Cukurova I. Relationship between lacrimal bone thickness and lacrimal sac in chronic dacryocystitis. J Craniofac Surg. 2020;31:207–9. https://doi.org/10.1097/ SCS.0000000000005856. 116. Mafee MF, Haik BG.  Lacrimal gland and fossa lesions: role of computed tomography. Radiol Clin N Am. 1987;25:767–79. 117. Kumar VA, Esmaeli B, Ahmed S, Gogia B, Debnam JM, Ginsberg LE. Imaging features of malignant lacrimal sac and nasolacrimal duct tumors. AJNR Am J Neuroradiol. 2016;37:2134–7. https:// doi.org/10.3174/ajnr.A4882. 118. El-Sawy T, Frank SJ, Hanna E, Sniegowski M, Lai SY, Nasser QJ, et al. Multidisciplinary management of lacrimal sac/nasolacrimal duct carcinomas. Ophthalmic Plast Reconstr Surg. 2013;29:454– 7. https://doi.org/10.1097/IOP.0b013e31829f3a73. 119. Sakaida H, Kobayashi M, Yuta A, Imanishi Y, Majima Y. Squamous cell carcinoma of the nasolacrimal duct. Eur Arch Otorhinolaryngol. 2009;266:455–8. https://doi.org/10.1007/ s00405-­008-­0677-­x. 120. Ni C, D’Amico DJ, Fan CQ, Kuo PK.  Tumors of the lacrimal sac: a clinicopathological analysis of 82 cases. Int Ophthalmol Clin. 1982;22:121–40. https://doi. org/10.1097/00004397-­198202210-­00010. 121. Rahangdale SR, Castillo M, Shockley W.  MR in squamous cell carcinoma of the lacrimal sac. AJNR Am J Neuroradiol. 1995;16:1262–4. 122. Natarajan A, Chandra P, Purandare N, Agrawal A, Shah S, Puranik A, et al. Role of fluorodeoxyglucose positron emission tomography/computed tomography in various orbital malignancies. Indian J Nucl Med. 2018;33:118–24. https://doi.org/10.4103/ijnm. IJNM_135_17.

117 123. Stefanyszyn MA, Hidayat AA, Pe’er JJ, Flanagan JC.  Lacrimal sac tumors. Ophthalmic Plast Reconstr Surg. 1994;10:169–84. https://doi.org/10.1097/00002341-­199409000-­00005. 124. Sjö LD.  Ophthalmic lymphoma: epidemiology and pathogenesis. Acta Ophthalmol. 2009;87:1–20. https://doi. org/10.1111/j.1755-­3768.2008.01478.x. 125. Palamar M, Midilli R, Ozsan N, Egrilmez S, Sahin F, Yagci A. Primary diffuse large B-cell lymphoma of the lacrimal sac simulating chronic dacryocystitis. Auris Nasus Larynx. 2011;38:643– 5. https://doi.org/10.1016/j.anl.2011.01.012. 126. Gao HW, Lee HS, Lin YS, Sheu LF. Primary lymphoma of nasolacrimal drainage system: a case report and literature review. Am J Otolaryngol. 2005;26:356–9. https://doi.org/10.1016/j. amjoto.2005.02.011. 127. Sjö LD, Heegaard S, Prause JU, Petersen BL, Pedersen S, Ralfkiaer E.  Extranodal marginal zone lymphoma in the ocular region: clinical, immunophenotypical, and cytogenetical characteristics. Invest Ophthalmol Vis Sci. 2009;50:516–22. https://doi. org/10.1167/iovs.08-­2539. 128. de Palma P, Ravalli L, Modestino R, Grisanti F, Casillo F, Marzola A.  Primary lacrimal sac B-cell immunoblastic lymphoma simulating an acute dacryocystitis. Orbit. 2003;22:171–5. https://doi. org/10.1076/orbi.22.3.171.15620. 129. Tsao WS, Huang TL, Hsu YH, Chen N, Tsai RK. Primary diffuse large B cell lymphoma of the lacrimal sac. Taiwan J Ophthalmol. 2016;6:42–4. https://doi.org/10.1016/j.tjo.2014.11.002. 130. Montalban A, Liétin B, Louvrier C, Russier M, Kemeny JL, Mom T, et al. Malignant lacrimal sac tumors. Eur Ann Otorhinolaryngol Head Neck Dis. 2010;127:165–72. https://doi.org/10.1016/j. anorl.2010.09.001. 131. Kavoussi SC, Levin F, Servat JJ.  Orbital extension of untreated lacrimal sac melanoma following dacryocystorhinostomy. Ophthalmic Plast Reconstr Surg. 2016;32:e76. https://doi. org/10.1097/IOP.0000000000000539. 132. Shao JW, Yin JH, Xiang ST, He Q, Zhou H, Su W. CT and MRI findings in relapsing primary malignant melanoma of the lacrimal sac: a case report and brief literature review. BMC Ophthalmol. 2020;20:191. https://doi.org/10.1186/s12886-­020-­01356-­6. 133. Matsuo T, Tanaka T, Yamasaki O. Lacrimal sac malignant melanoma in 15 japanese patients: case report and literature review. J Investig Med High Impact Case Rep. 2019;7:2324709619888052. https://doi.org/10.1177/2324709619888052. 134. Schaefer DP.  Acquired etiologies of lacrimal system obstructions. In: Cohen AJ, Mercandetti M, Brazzo B, editors. The lacrimal system: diagnosis, management, and surgery. 2nd ed. Cham, Switzerland: Springer International; 2015. p. 43–68. 135. Kassel EE, Schatz CJ.  Anatomy, imaging, and pathology of the lacrimal apparatus. In: Som PM, Curtin HD, editors. Head and neck imaging. 5th ed. St. Louis, MO: Elsevier-Mosby; 2011. p. 757–853. 136. Asheim J, Spickler E.  CT demonstration of dacryolithiasis complicated by dacryocystitis. AJNR Am J Neuroradiol. 2005;26:2640–1. 137. LeBedis CA, Sakai O. Nontraumatic orbital conditions: diagnosis with CT and MR imaging in the emergent setting. Radiographics. 2008;28:1741–53. https://doi.org/10.1148/rg.286085515. 138. Raslan OA, Ozturk A, Pham N, Chang J, Strong EB, Bobinski M.  A comprehensive review of cross-sectional imaging of the nasolacrimal drainage apparatus: what radiologists need to know. AJR Am J Roentgenol. 2019;213:1331–40. https://doi. org/10.2214/AJR.19.21507.

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Orbit J. Matthew Debnam, Jiawei Zhou, and Bita Esmaeli

Orbital lesions in both children and adults comprise a wide range of both benign and malignant tumors. The benign tumors include schwannomas and neurofibromas in patients with neurofibromatosis. Malignant tumors include lymphoma, rhabdomyosarcoma, and metastases. Other lesions such as idiopathic orbit inflammation, sarcoidosis, Graves’ eye disease, and vascular lesions such as venous malformations may mimic orbital tumors both clinically and radiographically. Knowledge of the imaging features of these and other orbital tumors and diseases is important for patient care and to avoid permanent symptoms such as vision loss and other consequences. The role of the radiologist is to assess the imaging appearance of an orbital lesion which may aid in narrowing the differential diagnosis, comment on benign versus malignant radiographic features, and describe the pattern and extent of disease spread including outside of the orbit and any involvement of the head and neck region. Specific imaging features to be mentioned in the radiology report include the size of the lesion, whether the lesion is well-circumscribed or infiltrative, whether there is mass effect upon the globe, the location in the intraconal or extraconal space, and the presence of bone remodeling or destruction of the orbital walls. In addition, the radiologist should assess specific ocular or periocular structures that are involved including the extraocular muscles, optic nerve, lacrimal gland, and orbital foramen. The imaging modalities used for the evaluation of orbit include CT, MRI, PET/CT, and ultrasound. These modalities provide important information about staging, pre-surgical planning, and treatment response. CT aids in the delineation

of tumor extent and bone remodeling or destruction. MRI can also be used to evaluate the features of the tumors including soft tissue characteristics, sinonasal and intracranial involvement, and perineural spread. PET/CT provides information for assessing a tumor’s metabolic activity, detecting local and distant metastases, staging, determining a site for biopsy based on metabolic activity, and evaluating treatment response. Ultrasound is used to evaluate the parotid gland and neck for adenopathy and to guide fine-needle aspiration and core needle biopsy. The purpose of this chapter is to describe the demographics and imaging appearance of common and uncommon malignancies and diseases of the orbit. This is accomplished with a review of the disease background, clinical presentation, and imaging features on various modalities and should provide the radiologist with a means to narrow their differential diagnosis. Tumors arising from the globe and cranial nerves, including the optic nerve, and those occurring secondarily from the bone and sinonasal cavities are discussed in other chapters.

Anatomy Figure 4.1 shows schematic and imaging anatomy of the orbit. • Retrobulbar orbit is located posterior to the globe. • Divided into three compartments:

J. M. Debnam (*) Department of Neuroradiology, Division of Diagnostic Imaging, The University of Texas MD Anderson Cancer Center, Houston, TX, USA e-mail: [email protected] J. Zhou · B. Esmaeli Department of Plastic Surgery, Division of Surgery, The University of Texas MD Anderson Cancer Center, Houston, TX, USA e-mail: [email protected]; [email protected] © The Author(s), under exclusive license to Springer Nature Switzerland AG 2023 J. M. Debnam (ed.), Imaging Atlas of Ophthalmic Tumors and Diseases, https://doi.org/10.1007/978-3-031-17479-7_4

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Fig. 4.1  Orbital anatomy. (a) Schematic drawing of the orbit demonstrating the intraconal and extraconal spaces. (b) Axial T1 non-contrast MRI without fat saturation shows the extraocular muscles (white arrows), optic nerve (black arrow), intraconal space (thin black arrows), extraconal space (thin white arrows), and the expected location or the

orbital septum (dotted white arrow). (c) Coronal T1 post-contrast MRI with fat saturation shows the extraocular muscles (white arrow), optic nerve (black arrow), intraconal space (thin black arrow), and extraconal space (thin white arrow)

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–– The muscle cone consists of the extraocular muscles excluding the inferior oblique muscle. –– Intraconal space lies within the muscle cone. –– Extraconal space lies outside the muscle cone. • Extraocular muscles originate from the tendinous annulus of Zinn at the orbital apex and insert on the globe. • Orbital septum: fibrous tissue originating from the periosteum of the orbital rim that blends superiorly with the tendon of the levator palpebrae superioris muscle and inserts inferiorly into the dense connective tissue of the eyelids (tarsal plates). Barrier between the preseptal and postseptal spaces.

Lymphoma Figures 4.2, 4.3, and 4.4 show cases of orbital lymphoma.

Background • Most common malignancy of the orbit [1, 2]. • Type of non-Hodgkin lymphoma, mostly of the B-cell phenotype. • Four subtypes: extranodal marginal zone B-cell lymphoma (EMZL), diffuse large B cell lymphoma (DLBCL), follicular lymphoma (FL), and mantle cell lymphoma (MCL). • Most common subtype is EMZL. • EMZL and FL have a better prognosis than DLBCL and MCL [3].

Presentation • Usually present in adults without a gender bias. • Most common presenting symptoms: periorbital soft tissue swelling, mass, and proptosis. • Other symptoms: epiphora (excessive tearing), pain, and diplopia [3].

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Imaging • Lymphoma usually molds around or encases adjacent orbital structures rather than causing mass effect. • Bone destruction is rare; when present, these findings suggest an aggressive histology. • Occasionally, isolated extraocular muscle involvement or diffuse orbital infiltration may be seen [4].

CT • Hyperdense mass [4] with homogeneous enhancement [5].

MRI • T1 and T2 signal iso- to hypointensity reflective of high cellularity. • Avid enhancement following contrast administration [4]. • Restricted diffusion is often present on diffusion-­ weighed imaging (DWI) [6].

PET • High18F-FDG uptake is noted on PET studies except for low-grade MALT lymphoma, which can show low 18F-­ FDG uptake [7]. • Whole-body PET/CT is used for staging orbital lymphomas and detection of systemic metastases [8].

Key Points • Comment on other sites of involvement.

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Fig. 4.2  A 68-year-old female with follicular lymphoma, left proptosis, and pain. (a) Axial T2 MRI with fat saturation shows a homogeneously isointense appearance of follicular lymphoma in the superior left orbit (arrow). (b, c) Axial and coronal T1 post-contrast MRI with fat saturation shows homogeneous enhancement of the

well-circumscribed, lobulated mass (arrows). Note the molding around the globe (*) that is displaced inferiorly. (d) Axial 18F-FDG PET/CT shows FDG avidity of the left superior orbital lymphoma (arrow)

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Fig. 4.3  A 51-year-old male with low-grade B cell lymphoma and left orbital proptosis. (a) Axial T1 post-contrast MRI with fat saturation shows a homogeneously enhancing intraconal mass in the left orbit (arrows). Note the enhancement of the left sphenoid bone (small arrow) (b) Axial 18F-FDG PET/CT shows FDG avidity of the left

intraconal mass (arrow). (c) DWI shows a hyperintense signal in the left intraconal mass and sphenoid bone (arrows). (d) Apparent diffusion coefficient map shows corresponding hypointense signal in keeping with restricted diffusion of the lymphoma involving the left intraconal space and sphenoid bone (arrows)

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Fig. 4.4  A 56-year-old female with visual field defects and proptosis from orbital lymphoma. (a) Axial T1 non-contrast MRI without fat saturation shows infiltrative soft tissue in the intraconal and extraconal spaces in the left and right orbits (arrows). (b) Axial T2 MRI with fat saturation shows an isointense infiltrative appearance of lym-

phoma in the orbits (arrows). (c, d) Axial and coronal T1 post-contrast MRI with fat saturation show enhancing infiltrative lymphoma in the intraconal and extraconal spaces with enlargement of the EOMs (arrows)

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Leukemia Figures 4.5 and 4.6 show cases of orbital leukemia.

Background • Leukemia is a disorder of hematopoietic stem cells in the bone marrow [9]. • Lymphoid leukemia affects white blood cells (too many lymphocytes). • Myeloid leukemia affects cells that give rise to white blood cells (other than lymphocytes), red blood cells, and platelets. • Extramedullary deposits of lymphocytic and myelogenous precursor cells can be found in any organ [10]. • Ocular manifestations occur more commonly with myeloid leukemia as opposed to lymphoid leukemia [11, 12] and most commonly involve the retina [9]. • Granulocytic sarcoma, also known as myeloid sarcoma and previously as chloroma, is a solid tumor of primitive granulocytes, vascular stroma, and connective tissue occurring in patients with myelogenous leukemia [13]. • Orbital granulocytic sarcoma is rare in adults and more commonly present in pediatric patients [14].

Presentation

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• Usually encases rather than invading structures. • May also appear as infiltrative disease. • Bone erosion, demineralization, and periosteal reaction occur less commonly. • Granulocytic sarcoma usually presents as a soft-tissue mass in the lateral orbital wall that may have infiltrative borders [13].

CT • Isointense or slightly hyperintense and demonstrates mild homogeneous enhancement [10]. • There may be invasion of the orbital fat and extension to involve the eyelid. • Calcification is not present. • Granulocytic sarcomas are homogeneously isodense to slightly hyperdense with homogeneous enhancement [13].

MRI • T1 slightly hyperintense and T2 isointense [17]. • Granulocytic sarcoma is T1 iso- to hypointense, T2 heterogeneously iso- to hyperintense, and homogeneously enhancing [13].

• The globes and orbits are the third most common site after the meninges and testes [15]. • Ocular involvement can precede the diagnosis or can occur during the disease [16]. • Central nervous system (CNS) involvement may include cranial nerve infiltration and palsies as well as papilledema [9].

PET

Imaging

• Comment on other sites of involvement.

• Homogenous mass that molds to the orbit [10].

• Orbital leukemia is 18F-FDG- avid. PET/CT is sensitive for the detection of granulocytic sarcoma and additional sites of disease [18].

Key Points

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Fig. 4.5 (a) An 82-year-old female with acute myeloid leukemia who presented with diplopia, pain, and restriction of movement of the globes. (a) Axial T1 post-contrast MRI with fat saturation shows infiltrative disease in the orbital apex bilaterally (arrows). (b, c) A

78-year-old female with relapsed acute myeloid leukemia. (b, c) Axial and coronal T1 post-contrast MRI with fat saturation show solid, homogeneously enhancing granulocytic sarcoma in the superior right orbit and superior rectus-levator muscle complex (arrows)

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Fig. 4.6  A 25-year-old male with T-cell acute lymphoid leukemia who presented with right periorbital swelling. (a) Axial CT with contrast, soft tissue window shows extensive bilateral neck adenopathy (arrows). (b) Coronal CT with contrast, soft tissue window shows enlargement of bilateral lacrimal glands (white arrows) with disease in the superior orbits (black arrows) and the presence of right perior-

bital soft tissue infiltration (thin white arrow). (c) Axial T1 post-­ contrast MRI with fat saturation shows enlargement of bilateral lacrimal glands (arrows) and bilateral periorbital soft tissue infiltration (white arrows). (d) Coronal T1 post-contrast MRI with fat saturation shows enlargement of bilateral lacrimal glands (arrows) and disease in the superior orbits (thin arrows)

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Rhabdomyosarcoma Figure 4.7 shows cases of orbital rhabdomyosarcoma.

Background • RMS is a tumor of striated muscle or mesenchymal cell precursors [19, 20].

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• Most common childhood malignancy of the orbit, accounting for 10% of all cases of RMS [21, 22]. • Two most commonly encountered subtypes: embryonal and alveolar. • Orbital RMS occurs less frequently in the adult population [23]. • RMS may arise from adjacent structures, including the sinonasal cavity, and spread to the orbit [24].

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Fig. 4.7 (a, b) A 6-year-old male with left orbital embryonal rhabdomyosarcoma. (a) Axial T2 MRI with fat saturation shows a homogeneously hyperintense mass in the inferior left orbit (arrow). (b) Sagittal T1 post-contrast MRI with fat saturation shows homogeneous enhancement of the well-circumscribed left inferior orbital mass (arrow). (c, d) A 13-year-old female with left orbital swelling due to an

embryonal rhabdomyosarcoma. (c) Axial T2 MRI with fat saturation shows a heterogeneously isointense mass in the superior left orbit (arrow). (d) Axial T1 post-contrast MRI with fat saturation shows heterogeneous enhancement of the left superior orbital rhabdomyosarcoma. Note small rings of enhancement within the mass (arrow)

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Presentation

Schwannoma

• In the pediatric population, RMS presents with visible signs such as an orbital mass, periorbital soft tissue swelling, proptosis, or blurry vision. • In the adult population, secondary signs such as sinus congestion and infection are more common than an orbital or sinonasal mass [25].

Figures 4.8 and 4.9 show cases of orbital schwannomas.

Imaging • Usually appears as a soft-tissue mass. • Imaging features similar to other soft tissue sarcomas [23]. • Calcification, hemorrhage, and necrosis are uncommon [24]. • Eyelid thickening and bone thinning or destruction in up to 40% of cases. • RMS may extend into adjacent paranasal sinuses or intracranially. • Predictors of poor outcome: tumor invasiveness, regional lymph node involvement, metastasis, and older age at diagnosis [25].

CT • Orbital RMS appears as a well-circumscribed mass that is isodense to muscle with moderate to marked enhancement [26].

MRI • T1 isointense and T2 hyperintense to muscle. • Moderate to marked and homogeneous to heterogeneous enhancement [26]. • Thin rings of enhancement have been described [24].

Background • Benign nerve sheath tumors comprised of spindle cells arranged as compact (Antoni type A) or loose (Antoni type B) tissues [27]. • The more commonly involved nerves about the orbit are the trigeminal (CN V) and facial (CN VII) nerves. • Soft tissue sarcomas that originate from the peripheral nerve sheath are termed malignant peripheral nerve sheath tumors (MPNSTs) [28].

Presentation • Denervation-induced muscle atrophy or a sensory deficit can aid in identifying the cranial nerve of origin or occurs if an adjacent cranial nerve is affected from long-­ standing mass effect [29]. • Clinical symptoms that suggest transformation to an MPNST: non-specific symptoms including new pain, increased growth, and new neurologic deficits [30].

Imaging • Slow growth of a schwannoma is characterized by smooth expansion of an affected neural foramen, bone remodeling, and/or mass effect on adjacent soft tissues [29, 31].

CT • Cranial nerve schwannomas demonstrate variable enhancement [31, 32].

Key Points • Try to determine the primary location of the tumor, e.g., in the orbit or sinuses. • Assess for regional lymph node involvement.

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Fig. 4.8  A 65-year-old male with left exophthalmos due to an orbital schwannoma. (a) Axial CT with contrast, bone window shows subtle remodeling of the left orbital floor (arrow) adjacent to a mass (thin arrow). (b) Axial T2 MRI with fat saturation shows a heterogeneously

hyperintense, well-circumscribed schwannoma in the inferolateral left orbit (arrow). (c, d) Axial and sagittal T1 post-contrast MRI with fat saturation show heterogeneous enhancement of the schwannoma (arrows)

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Fig. 4.9  A 34-year-old female with an incidentally discovered left orbital schwannoma. (a) Axial T1 non-contrast MRI without fat saturation shows a well-circumscribed homogeneous appearance of the left orbital schwannoma (arrows). (b) Coronal T2 MRI with fat saturation shows a hyperintense appearance of the schwannoma (arrow)

with superotemporal displacement of the optic nerve (thin arrow). (c, d) Axial and sagittal T1 post-contrast MRI with fat saturation show homogeneous enhancement of the schwannoma (arrow) that is narrowed as it passes through the superior orbital fissure (thin arrow)

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MRI • Appearance depends on the components of Antoni type A and B tissues [27]. • T1 hypo- to isointense with avid enhancement. • T2 heterogeneously hyperintense due to compactly arranged cells (Antoni type A pattern-hypointense) intermixed with areas of loosely arranged cells (Antoni type B pattern-hyperintense) with variable water content and cellularity [27, 33] thus can be hypo- to isointense [34]. • Larger lesions may demonstrate heterogeneous enhancement, internal cysts, and hypointense foci of hemosiderin related to internal hemorrhage [29]. • MRI findings of MPNSTs: larger size, heterogeneous signal and enhancement, internal necrosis without enhancement, irregular margins, and local invasion [34]. • Restricted diffusion associated with MPNSTs has been described [34]; however, further work on this topic is needed [28].

PET • In addition to intense18F-FDG avidity, both schwannomas and MPNSTs may have a large size and demonstrate a heterogeneous appearance [34].

Key Points • Attempt to determine which nerve is involved. • Describe orbital foraminal involvement e.g., superior orbital fissure, foramen rotundum.

Solitary Fibrous Tumor Figures 4.10, 4.11, and 4.12 show cases of orbital solitary fibrous tumors.

Background • A solitary fibrous tumor (SFT) , formerly named hemangiopericytoma [35], is a spindle-cell neoplasm that originates from mesenchymal tissue and most frequently occurs in the pleura [36]. • Increasing incidence has been noted in extra-pleural sites, including the head and neck, chest, abdomen, pelvis, and meninges [37, 38].

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• SFTs may occur in the postseptal orbit, lacrimal gland and sac, and lower eyelid [36, 37].

Presentation • Wide age range: from 9 to 76 years; no gender predilection [39, 40]. • Symptoms: slowly progressive exophthalmos or palpable mass [41].

Imaging • SFTs present as well-defined, ovoid masses. • More common in the superolateral or superomedial orbit followed by the inferomedial orbit [42].

CT • SFTs are isodense to slightly hyperdense on non-­ contrast CT [42, 43]. • Rapid enhancement following contrast administration [42].

MRI • Generally T1 homogeneously isointense [43]. • T1 hypointense components are related to cystic or myxoid degeneration [44]. • T2 isointense to hypointense related to fibrous tissue with a large collagen content. • Areas of fresh fibrosis, internal hemorrhage, and cystic degeneration may be T2 hyperintense [42]. • Signal flow voids from vessels may be noted [43]. • Areas of T2 hyperintensity with strong enhancement have been described as suggestive of an SFT [45]. • Rapid enhancement with a washout pattern of contrast may aid in diagnosis [42]. • Slight restricted diffusion has been reported [41].

Key Points • T2 hypointense with rapid uptake of contrast. • Signal flow voids from vessels may be present.

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Fig. 4.10  A 39-year-old male with left proptosis due to a solitary fibrous tumor. (a) Axial CT with contrast, bone window shows a mass in the left posterior orbit with widening of the superior orbital fissure (arrow). (b) Axial T2 MRI with fat saturation shows a heteroge-

neous, predominately hypointense mass in the left orbital apex (arrow). (c, d) Axial and coronal T1 post-contrast MRI with fat saturation shows small hypointense vascular flow voids (arrows) coursing through the homogeneously enhancing mass

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Fig. 4.11  A 65-year-old male with right proptosis and orbital pain related to a solitary fibrous tumor. (a) Axial T2 MRI with fat saturation shows a right orbital heterogeneous intraconal mass with hypo- to isointense signal and multiple vascular flow voids (arrows) and proptosis of the globe. (b) Axial T1 post-contrast MRI with fat saturation shows heterogeneous enhancement of the tumor with multiple vascular flow voids (arrows)

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Fig. 4.12  An 8-year-old male with right lower eyelid swelling after trauma and an incidental solitary fibrous tumor. (a, b) Axial T2 MRI with fat saturation shows a well-circumscribed intraconal mass with iso- to hyperintense signal (arrows). (c, d) Corresponding Axial T1

post-contrast MRI with fat saturation shows more solid enhancement in the regions of the tumor that are hyperintense on the T2-weighted series (arrows)

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Liposarcoma Figure 4.13 shows a case of an orbital liposarcoma.

Background

• Five histologic subtypes: well-differentiated, dedifferentiated, myxoid, pleomorphic, and mixed [46–48]. • The majority occur in the deep soft tissues of the retroperitoneum and extremities with a small percentage occurring in the head and neck [49].

• Tumor with differentiation of malignant lipoblasts [46, 47].

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Fig. 4.13  A 44-year-old male with a right orbital liposarcoma. (a) Axial T1 non-contrast MRI without fat saturation shows a hyperintense mass in the superonasal right orbit (arrows). (b, c) Axial and coronal T1 post-contrast MRI with fat saturation shows enhancement

of the solid component of the liposarcoma (arrows) and suppression of fat signal in the fatty component (thin arrow). (d) Axial 18F-FDG PET/CT shows FDG avidity of the solid component of the liposarcoma (arrow)

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Presentation

Orbital Metastases

• More common in males in the fourth through sixth decades. • Painless soft tissue mass [46, 47].

Figures 4.14, 4.15, 4.16, 4.17, 4.18, and 4.19 show cases of orbital metastases.

Imaging

Background

• Hypodense component similar to a lipoma with variable soft tissue densities. • Intratumoral calcification and internal bleeding lead to a hyperdense appearance [46, 50].

• Tumors that metastasize to the obit represent between 2.5 and 10% of all orbital mass lesions [52]. • Approximately 35% of patients with an orbital metastasis do not have a history of a primary tumor at the time of diagnosis [53]. • The most common primary tumor sites are the breast, lung, prostate, and malignant melanoma arising from the skin [54, 55]. • In children, orbital metastases arise from metastatic neuroblastoma, Wilms’ tumor, and Ewing’s sarcoma [56].

MRI

Presentation

• Liposarcomas contain fat similar to lipomas, with variable amounts of associated soft tissue.

CT

• Well-differentiated liposarcoma appears similar to a • Symptoms: proptosis, diplopia, decreased vision, eyelid benign lipoma with T1 hyperintense and T2 iso- to swelling, conjunctival swelling (chemosis), and redness hypointense signal and minimal to no contrast [57]. enhancement. • Less well-differentiated subtypes (pleomorphic, myxoid) show T1 hypointense tissue with septa and scat- Imaging tered islands of fatty T1 hyperintense tissue. • In these cases, the solid components of the mass are T2 • Metastases can be intraconal or extraconal [58, 59] or iso- to hyperintense with an iso- to hypointense appearinvolve the extraocular muscles (EOMs) with enlargeance of the fatty tissue. ment of the involved muscle [60]. • Stromal enhancement is present in less well-­ • Variety of appearances from nodular to diffusely differentiated subtypes [51]. infiltrative. • Single or multiple; usually unilateral but can be bilateral [57]. PET • Extension into the intracranial compartment or paranasal sinuses may also occur. • Well-differentiated liposarcomas show low 18F-FDG avidity reflective of a low malignant potential while less well-differentiated subtypes show marked 18F-FDG CT uptake [49]. • Isodense on non-contrast CT and demonstrates enhancement.

Key Points

• Search for soft tissue associated with the fat component.

136 Fig. 4.14  A 49-year-old female with carcinoma of the anal canal who presented with left periorbital erythema and ophthalmoplegia (weakness of the EOMs). (a, b) Axial and coronal CT with contrast, soft tissue window shows a solid metastasis in the posterior superior left orbit (arrows) with soft tissue standing in the anterior left orbital and periorbital soft tissues from unrelated cellulitis (thin arrow)

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Fig. 4.15  A 61-year-old male with melanoma who presented with right proptosis and diplopia. (a) Axial T1 non-contrast MRI without fat saturation shows an isointense appearance of the right orbital mass (arrow). (b) Axial T2 MRI with fat saturation shows an isointense right orbital metastasis (arrow). (c) Axial T1 post-contrast MRI with fat saturation demonstrates heterogeneous enhancement of the orbital

melanoma (arrow). (d) DWI shows hyperintense signal in the right orbital melanoma (arrow). (e) Apparent diffusion coefficient map shows corresponding hypointense signal in the mass consistent with restricted diffusion (arrow). (f) Axial T1 post-contrast MRI without fat saturation shows a small right cerebellar metastasis (arrow)

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Fig. 4.16  A 74-year-old male with melanoma and left proptosis. (a) Axial T1 non-contrast MRI without fat saturation shows an isointense intraconal metastasis in the left orbit (arrow). (b) Axial T2 MRI with fat saturation shows predominately hyperintense signal in the metastasis (arrow). (c, d) Axial and coronal T1 post-contrast MRI with fat

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Fig. 4.17  A 42-year-old male with recurrent glomus tumor, left orbital pain, and vision loss. (a) Axial T1 post-contrast MRI with fat saturation demonstrates an intraconal and extraconal left orbital mass with extension to the orbital apex and into the ethmoid air cells (arrow). Note metastases involving the sphenoid bones bilaterally (thin

saturation show peripheral enhancement (arrows). (e) DWI shows hyperintense signal in the lesion (arrow). (f) Apparent diffusion coefficient map shows corresponding hypointense signal in the metastasis in keeping with restricted diffusion (arrow)

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arrows). (b) Coronal T1 post-contrast MRI with fat saturation shows the intraconal and extraconal left orbital mass and a right frontal calvarial metastasis (arrows). Note inferolateral displacement of the optic nerve (thin arrow). (c) Axial CT without contrast, lung window shows numerous pulmonary metastases

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Fig. 4.18  A 63-year-old male with renal cell carcinoma who presented saturation shows a heterogeneous hypo- to isointense appearance of with right proptosis due to an orbital metastasis. (a) Axial T1 non-­ the metastasis (arrow). (c) Axial T1 post-contrast MR with fat saturacontrast MRI without fat saturation shows an isointense metastasis in tion shows homogeneous enhancement of the metastasis (arrow) the right lateral rectus muscle (arrow). (b) Axial T2 MRI with fat

Fig. 4.19  A 23-year-old female with breast cancer who presented with orbital pain and diplopia. (a, b) Axial and coronal T1 post-contrast MRI with fat saturation show metastases to bilateral medial rectus muscles (arrows) with intraconal disease in the right orbit surrounding the optic nerve (thin arrows)

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MRI

Key Points

• The appearance depends on the tumor type, but metastases tend to be T1 hypointense, T2 hyperintense with variable enhancement. • T1 hyperintense lesions are from vascular metastasis such as renal or thyroid carcinoma, or melanoma [61].

• List the number of orbital lesions. • Describe the orbital structures that are involved, e.g., EOM, bone. • Search for other sites of involvement, e.g., brain metastases, neck nodes. • Attempt to determine if the lesion is a metastasis versus a separate primary tumor.

PET •

Graves’ Eye Disease

F-FDG uptake depends on the FDG avidity of the primary tumor [62]. 18

Figures 4.20 and 4.21 show cases of thyroid eye disease.

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Fig. 4.20 (a, b) A 66-year-old female with a history of hyperthyroidism who presented with right eye exophthalmos and intermittent diplopia due to Graves’ eye disease. (a) Coronal T1 post-contrast MRI with fat saturation shows enlargement with enhancement of the right superior, medial, and inferior rectus muscles (arrows). (b) Axial T1 post-­ contrast MRI with fat saturation shows enlargement of the right medial rectus muscle that spares the tendinous insertions (arrows).

(c, d) A 43-year-old female with hyperthyroidism and Graves’ eye disease. (c) Coronal T1 post-contrast MRI with fat saturation shows enlargement of the left superior rectus-levator muscle complex (arrow). (d) Coronal T2 MRI with fat saturation shows enlargement and signal hyperintensity in the left superior rectus-levator muscle complex related to edema (arrow)

140 Fig. 4.21  A 30-year-old female with a history of hyperthyroidism and Graves’ eye disease. (a, b) Axial and coronal T1 post-contrast MRI with fat saturation shows enlargement of the EOMs with crowding at the orbital apex and compression upon the optic nerves (arrows)

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Background • Graves’ disease is an autoimmune disorder. • Graves’ disease is generally attributed to genetic (79%) or environmental factors (21%) [63]. • Circulating anti-thyroid-stimulating hormone receptor autoantibodies bind to thyroid-stimulating hormone receptors, stimulating the thyroid follicular cells to release T3 and T4. • Increased T3 and T4 cause thyrotoxicosis with autoreactive lymphocyte deposition in the thyroid gland [64]. • Graves’ eye disease (GED): an inflammatory condition affecting the orbital soft tissues. –– Approximately 25% of patients with Graves’ disease have GED [65, 66].

Presentation • More common in women. • Usually presents between 30 and 60 years of age [67]. • Symptoms of hyperthyroidism: fatigue, weight loss, palpitations, anxiety, sleep disturbance, heat intolerance, and polydipsia [68, 69]. • Upper eyelid retraction occurs in greater than 80% of patients with GED. –– Eyelid retraction may be recognized by others [70].

Imaging

–– Occurs in approximately 6% of patients [71]. –– May arise from hypertrophied EOMs compressing on the optic nerve at the orbital apex. • Bilateral orbital involvement in approximately 90% of patients even if clinical manifestations appear unilateral or asymmetric [72]. • Enlarged EOMs with sparing of the tendinous insertions [72]. • Signs of CON: EOM crowding at the orbital apex and/or fat plane effacement around the optic nerve by enlarged EOMs [73–77] and optic nerve narrowing [78].

CT • Hypodensity of the EOMs from lymphocyte accumulation and mucopolysaccharide deposition [72]. • Increased orbital fat may lead to stretching of the optic nerve, eyelid edema, lacrimal gland prolapse, and bony orbit remodeling [79].

MRI • Fatty infiltration of the EOMs appears T1 hyperintense. • T2 hyperintensity of EOMs related to edema. • Enlargement and enhancement of the EOMs [80, 81].

Key Points

• Proptosis results from expansion of retro-orbital fat and/or EOMs. • Compressive optic neuropathy (CON): serious but relatively infrequent complication.

• Search for the presence or absence of EOM tendon involvement.

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• Describe involved muscles (lateral rectus is rarely involved in isolation for GED). • Search for signs of optic nerve compression at the orbital apex by enlarged extraocular muscles.

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Idiopathic Orbital Inflammation Figures 4.22 and 4.23 show cases of idiopathic orbital inflammation.

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Fig. 4.22  A 35-year-old male with proptosis and periorbital edema due to idiopathic orbital inflammation. (a) Axial T2 MRI with fat saturation shows isointense intraconal disease in the right orbit and an isointense mass involving the left medial rectus muscle (arrows). (b) Axial T1 post-contrast MRI with fat saturation shows enhancing disease in the left medial rectus muscle (arrow) with involvement of

the tendinous insertion (thin arrow). Note enhancing disease in the right intraconal space. (c) Coronal T1 post-contrast MRI with fat saturation show enhancing disease in the right intraconal space (arrow) with abutment of the optic nerve (thin arrow). The left medial rectus muscle is also enlarged. (d) Axial T1 post-contrast MRI with fat saturation shows decreasing disease following treatment

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Fig. 4.23 (a, b) An 18-year-old male presented with gradual vision loss in the right eye due to idiopathic orbital inflammation (IOI) . (a) Axial T1 post-contrast MRI with fat saturation shows disease in the right intraconal and extraconal spaces with involvement of the lateral rectus muscle (arrows) and right masticator space (thin arrow). (b) Coronal T1 post-contrast MRI with fat saturation shows disease in the right intraconal and extraconal spaces, involving the extraocular muscles, right maxillary sinus (arrows), and the right masticator

space (arrows). (c, d) A 63-year-old male with recurrent IOI. (c) Axial T1 post-contrast MRI with fat saturation shows disease in the right lateral rectus muscle and intraconal space with posterior extension to the cavernous sinus (arrows). (d) Coronal T1 post-contrast MRI with fat saturation shows disease in the right lateral rectus muscle and intraconal space (arrows) that surrounds and elevates the optic nerve (thin arrow)

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Background

Key Points

• Idiopathic orbital inflammation (IOI) was previously referred to as orbital pseudotumor. • Inflammatory conditions characterized by polymorphous lymphocyte infiltration and fibrosis of varying degrees [82, 83]. • Third most common disease affecting the orbit after GED and lymphoproliferative disorders [84]. • Tolosa–Hunt syndrome: a rare subtype of IOI with involvement confined to the orbital apex and/or cavernous sinus, resulting in acute orbital pain and paralysis of cranial nerves III, IV, and VI [85].

• Search for the presence or absence of EOM tendon involvement. • Determine which muscles are involved (lateral rectus is rarely involved in isolation for GED). • Search for involvement of the orbital apex and/or cavernous sinus.

Presentation

Background

• Symptoms: headache, periorbital pain, and inflammatory signs including soft tissue swelling and erythema. • Compression upon the orbital apex and cavernous sinus involvement may lead to decreased visual acuity and cranial nerve palsies [86].

• Immunoglobulin G4-related disease (IgG4-RD) is a systemic disease of unknown etiology. • Characterized by inflammation, fibrosis, and tissue infiltration with plasma cells that express IgG4. • Various organs may be involved, including the pancreas, bile duct, liver, retroperitoneal soft tissues, lung, thyroid, salivary glands, and lymph nodes either alone or systematically [89, 90]. • After the pancreas, the head and neck region is the second most affected site [91].

Imaging • Nonspecific inflammatory soft tissue is present in the orbit with infiltration of the orbital fat. • When the EOMs are involved, IOI may include the tendinous portion of the muscles. • Other sites of involvement include the lacrimal gland, optic nerve including the junction with the globe, and adjacent periorbital soft tissues. • Retro-orbital involvement may occur from extension through the superior and inferior orbital fissures and the optic canal with involvement of the cavernous sinus [82].

CT • Enhancement with contrast [85].

MRI • IOI is T1 isointense and T2 iso- to hypointense which may be due to fibrosis. • Variable contrast enhancement [86, 87]. • Restricted diffusion has been described [88].

Immunoglobulin G4-Related Disease Figures 4.24 and 4.25 show cases of IgG4-related disease.

Presentation • Symptoms of IgG4-RD include hypophysitis, thyroiditis, pancreatitis, cholecystitis, retroperitoneal fibrosis, and lymphadenopathy [91–95]. • Occurs predominantly in older men. • Frequently associated with elevated serum IgG4 levels [92, 96]. • Can involve the orbit including the EOMs and optic nerve. • Other sites of disease in the head and neck: pituitary gland, cavernous sinus, paranasal sinuses, and cervical nodes [91]. • Lacrimal involvement (dacryoadenitis) may occur in isolation or with salivary gland involvement (sialadenitis) as part of Mikulicz’s disease (painless bilateral enlargement of the lacrimal, parotid, and submandibular glands) [94].

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Fig. 4.24  A 43-year-old male with right lacrimal swelling due to IgG4-RD. (a) Axial T1 post-contrast MRI with fat saturation shows infiltrative disease involving the right inferior rectus muscle with posterior spread of disease to the right cavernous sinus (arrows). (b) Axial T1 post-contrast MRI with fat saturation shows disease in the right premaxillary soft tissues, pterygopalatine fossa, right masticator space, and foramen ovale (arrows). (c) Coronal T1 post-contrast

MRI with fat saturation shows disease surrounding the right optic nerve and involving the right infraorbital nerve (V2) (arrows) and the superior rectus-levator muscle complex. (d) Coronal T1 postcontrast MRI with fat saturation shows disease involving the right mandibular nerve (V3) (arrows) as it extends through the foramen ovale (thin arrow)

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Fig. 4.25  A 60-year-old male with right lacrimal swelling due to IgG4-RD. (a) Axial T1 post-contrast MRI with fat saturation shows disease in the inferior right orbit (arrow) and the right pterygopalatine fossa (thin arrow). (b) Coronal T1 post-contrast MRI with fat saturation shows disease in the inferior right orbit with involvement of the

right infraorbital nerve (V2) (arrow) and right face (thin arrow). (c) Axial 18F-FDG PET/CT shows FDG avidity in the right infraorbital nerve (V2) (arrow). (d) Coronal 18F-FDG PET/CT shows FDG-avid disease along the right side of the thoracic vertebral bodies (arrows)

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Imaging

Key Points

• The most common sites are the lacrimal gland and EOMs, which are affected in most patients with this diagnosis [96]. • The lacrimal glands are enlarged, which may be unilateral or bilateral. • Other possible findings about the orbits include enlargement of the EOMs, infiltration of the orbital fat, cranial nerve involvement especially the infraorbital nerve (V2), and soft tissue in the cavernous sinuses and Meckel’s caves [96]. • Associated paranasal sinus inflammatory mucosal thickening has been reported [96].

• Search for involvement of the salivary glands, e.g., parotid, submandibular glands. • Search for involvement of cranial nerves, e.g., infraorbital nerve, cavernous sinus, and Meckel’s cave. • Search for other sites of disease involvement throughout the body.

CT

Background

• Homogeneous soft tissue with contrast enhancement.

• Chronic systemic multiorgan disorder characterized by a non-caseating granulomatous reaction. • Unknown etiology. • Can affect nearly every organ in the body, with orbital involvement reported in 25–83% of the patients [98, 99]. • The lungs, skin, orbits, and lymph nodes are most commonly affected; however, the liver, kidneys, heart, and brain may also be involved [98, 99]. • Biopsy is often required and demonstrates the non-­ caseating granulomas [4].

MRI • T1 hypointensity, T2 hypo- to hyperintensity with homogeneous enhancement [92, 95].

PET

Sarcoidosis Figures 4.26 and 4.27 show cases of sarcoidosis in the orbit.

• IgG4-RD cases are 18F-FDG-avid and PET/CT is useful to detect multi-organ involvement, guide biopsies, and assess treatment response [92, 97].

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Fig. 4.26  A 60-year-old female being treated for sarcoidosis presented with fullness in the right upper eyelid. (a, b) Axial and coronal CT with contrast, soft tissue window shows disease in the superonasal right

orbit (arrows). (c) Coronal CT with contrast, soft tissue window shows anterior extension of disease superior and medial to the right globe (arrow) with inferolateral displacement of the globe

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Fig. 4.27  A 57-year-old male presented with right eye swelling and diplopia due to sarcoidosis. (a) Axial T2 MRI with fat saturation shows hypointense disease in the pre- and postseptal superonasal right

orbit (arrow). (b) Axial T1 post-contrast MRI with fat saturation shows heterogeneous enhancement of the disease (arrow). (c) Plain chest radiograph shows bilateral hilar adenopathy (arrows)

Presentation

MRI

• Primarily develops in patients aged 25–45  years old, although children and older individuals may also be affected [100]. • Ocular involvement in 20% of patients [101]. • African Americans have a higher incidence of ocular involvement compared to Whites [102]. • Manifestations include anterior uveitis followed by posterior uveitis which can severely impact visual acuity [103]. • The cranial nerves, leptomeninges, brain parenchyma, and hypothalamic-pituitary axis may also be involved. Diabetes insipidus may result [100]. • The parotid gland is involved in 5% of cases [101]. • Other findings include enlarged hilar lymph nodes, pulmonary infiltrates, and skin disease. • High serum angiotensin-converting enzyme level is present but not specific [104].

• EOM involvement is characterized by abnormal thickening and enhancement of the muscles and tendinous insertions. • Infiltrative and enhancing soft tissue can be seen in the retrobulbar fat [107]. • The optic nerves may show thickened enhancing nodules with T2 signal hyperintensity of the nerve. • Diffusely enlarged and enhancing lacrimal glands [108].

Imaging • Can involve the orbital fat, EOMs, optic nerve sheath, and lacrimal gland [105]. • Displacement of the globe including proptosis may be present [106].

CT • Homogeneous enhancement [105].

Nuclear Medicine • PET/CT and Gallium scans aid in diagnosis when orbital, parotid and bilateral hilar uptake is detected [107].

Key Points • Search for other sites of involvement throughout the body (e.g., regional lymph nodes).

Granulomatosis with Polyangiitis Figures 4.28 shows a case of granulomatosis with polyangiitis.

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Fig. 4.28  A 37-year-old male with granulomatosis with polyangiitis who presented with left orbital pain. (a) Axial CT with contrast, soft tissue window shows a heterogeneously enhancing soft tissue in the left orbit with compression upon a proptotic globe and destruction of the sphenoid bone (arrows). Disease extends posteriorly to involve bilateral cavernous sinuses, Meckel’s caves, and the tentorium (thin

arrows). (b) Coronal CT with contrast, soft tissue window shows the intraconal and extraconal inflammatory soft tissue in the left orbit (arrow). Note changes in the sinonasal cavity, including perforation of the nasal septum (thin arrow) and destruction of the medial walls of the maxillary sinuses. (c) Axial CT with contrast, soft tissue window shows disease in the pterygopalatine fossae (arrows)

Background

• The orbits are involved in up to 58% of patients; ocular manifestations are non-specific [110, 112]. • Granulomatous disease can lead to an orbital inflammatory mass often with proptosis and optic nerve compression. • Severe orbital disease can cause optic nerve compression and blindness [113]. • A granulomatous pseudotumor involving the cranial nerves of the cavernous sinus may present as Tolosa– Hunt syndrome with painful ophthalmoplegia (weakness and paralysis of EOMs) [114].

• Granulomatosis with polyangiitis is an idiopathic autoimmune disease leading to necrotizing granulomatous vasculitis [109]. • Previously named Wegener’s granulomatosis. • Antibodies to neutrophil cytoplasmic antigens (ANCA) are present in approximately 80–90% of patients [110]. • Tissue necrosis with vasculitis of small to medium vessels. • Characteristically presents with disease in the nasal cavity, lungs, and kidney [111].

Imaging

Presentation • The average age at presentation is 40–55 years, with no gender differences [111]. • The main presenting symptoms are usually related to the upper respiratory tract and renal and nasal cavity disease [112]. • Other symptoms: diplopia, epistaxis, septal perforation, chronic sinusitis, chest pain, and hemoptysis. • Small vessel vasculitis causes symptoms such as conjunctivitis, scleritis, uveitis, retinitis, and optic neuritis.

• Commonly presents as an inflammatory infiltrate that molds around the orbital contour and may involve the adjacent paranasal sinuses [115]. • Growth of the inflammatory infiltrate leads to the formation of an orbital mass [116]. • When the sinonasal cavity is involved, imaging findings include mucosal thickening, bone erosion, and destruction [117].

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CT

• Multisystem disease process that can affect any organ with a variety of manifestations [118, 119].

• CT demonstrates cartilage and bone destruction with nasal septum perforation, diffuse swelling of EOMs, and orbital inflammatory pseudotumor.  resentation P • Slightly hyperdense to nasal mucosa on contrast-­ enhanced studies [112]. • Median age at diagnosis is 70 years old. • Higher occurrence rate in men and Whites [121, 122]. • Bing–Neel syndrome (BNS) represents CNS involveMRI ment of Waldenstrom macroglobulinemia with neoplastic cells [123–125]. • In the early stages of disease, mucosal inflammation and granulation tissue may have a similar appearance. • In later stages, the disease appears slightly T1 hypoin- Imaging tense, T2 hyperintense with homogeneous enhancement. • CNS disease: brain parenchyma, dura, leptomeninges, • Contour may be indistinct [109]. cranial nerves (mainly the optic nerve), and spinal cord [123–125]. • Two categories of CNS involvement: diffuse form and Key Points tumoral form. –– May be unifocal or multifocal [126]. • Search for the presence of scleritis or optic neuritis. • Retro-orbital collections of lymphoplasmacytoid cells • Search for the involvement of the nasal cavity/paranasal can cause reduced motility and proptosis [127]. sinuses and bone destruction. • Lacrimal gland, conjunctival, and vitreous involvement have also been reported [128].

Waldenstrom Macroglobulinemia Figure 4.29 shows macroglobulinemia.

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MRI Waldenstrom

• Lesions in the diffuse form are reported to be T1 hypointense and T2 hyperintense. • In the brain parenchyma, “ring-shaped or nodular Background enhancement, with or without surrounding edema” has been reported [124]. • Waldenstrom macroglobulinemia is a type of non-­ • Thickened enhancement of the cranial nerves, meninHodgkin lymphoma also named “lymphoplasmacytic ges, and spinal nerve roots [124]. lymphoma” [118, 119]. • Mature B-cell neoplasm with small lymphocytes show- PET/CT ing “plasmacytoid–plasma cell differentiation in the absence of features of other lymphoproliferative disor- • Bing–Neel syndrome has been reported to be 18F-FDG ders” [119, 120]. avid [129]. • Waldenstrom macroglobulinemia is diagnosed with bone marrow involvement of IgM-producing lymphoplasmacytic lymphoma.

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Fig. 4.29  A 59-year-old male presented with intermittent blurry vision and was later diagnosed with Waldenstrom macroglobulinemia. (a) Axial T2 MRI with fat saturation shows isointense lesions in the superior orbits (arrows). (b, c) Axial and coronal T1 post-contrast MRI

with fat saturation shows homogeneous enhancement of lesions in bilateral superotemporal orbits (arrows). (d) Coronal CT with contrast, soft tissue window shows extensive bilateral cervical adenopathy (arrows)

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Amyloidosis

Imaging

Figures 4.30 and 4.31 show cases of amyloidosis.

• CT may be more informative than MRI in differentiating periocular and orbital amyloidosis owing to the higher sensitivity of detecting calcifications and bony changes. • With orbital involvement, the mass may mold to the globe but can cause displacement. • EOM enlargement may also be present [134, 135].

Background • Amyloidosis is a disease of unknown etiology characterized by the deposition of a proteinaceous amyloid protein in tissues and organs. • Amyloid may be localized or systemic and can accumulate in all the body’s tissues [130, 131]. • Often diagnosed by tissue biopsy. • Samples undergo Congo red staining and are viewed with a polarized light where amyloid has an affinity for the Congo red and green birefringence [132].

CT • On CT, amyloid may appear as a homogeneous, slightly hyperdense soft tissue mass that may show calcifications. • Erosion of the orbital wall has been reported [134, 135].

Presentation

MRI

• Typically affects middle-aged patients [133]. • Deposition of amyloid can occur in multiple ocular sites, including the orbit, orbital adnexa, EOMs, lacrimal gland, eyelid, and conjunctiva. • Symptoms: eyelid swelling, discomfort, proptosis, hyperemia of the bulbar conjunctiva, subconjunctival hemorrhage, and lacrimal gland enlargement [132].

• T2 hypointense with heterogeneous contrast enhancement [130, 131].

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Fig. 4.30  A 55-year-old male was referred after biopsy of a left orbital mass demonstrating amyloidosis. (a) Axial T2 MRI with fat saturation shows hypointense disease in the medial left orbit and ethmoid air cells (arrows). (b) Axial T1 post-contrast MRI with fat saturation shows

PET • May be 18F-FDG-avid.

c

heterogeneous enhancement of the disease (arrows). (c) Axial 18F-­ FDG PET/CT shows FDG avidity of the disease in the medial left orbit

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Fig. 4.31  A 64-year-old female who presented with pain and periorbital amyloidosis. (a) Axial non-contrast MRI without fat saturation shows isointense disease in the pre- and postseptal orbits, including the right intraconal space (arrows). (b) Axial T2 MRI with fat saturation shows a hypointense appearance of amyloidosis (arrows). (c)

Axial T1 post-contrast MRI with fat saturation shows a small degree of enhancement of the disease (arrows) except in the right medial and left lateral rectus muscles that demonstrate enhancement (thin arrows)

Erdheim–Chester Disease Figure 4.32 shows a case of Erdheim–Chester disease.

• Symptoms: exophthalmos, bone pain, diabetes insipidus, cerebellar or pyramidal symptoms, cardiac dysfunction, and renal impairment [141].

Background

Imaging

• Non-Langerhans cell histiocytosis characterized by multi-organ disease involvement. • Specific immunohistochemical profile: histiocytes stain positive for CD68, negative for CD1a, and positive or negative for S100 [136].

• Retro-bulbar masses may develop secondary to histiocyte infiltration [140, 141]. • Meningeal thickening may mimic meningiomas. • Vertebral involvement [140]. • Bony medullary sclerosis [139].

Presentation

MRI

• Average age at diagnosis: 53 years; slight male predominance [136, 137]. • Multiple systemic manifestations including the CNS and cardiovascular, pulmonary, and musculoskeletal systems. • Predilection for appendicular skeleton [138]. • The hypothalamic-pituitary axis is the most common site in the CNS [139]. • Variable clinical course: some patients asymptomatic, and others succumb following rapid disease progression [140].

• Intraconal lesions are T1 and T2 hypointense and enhance [140, 141].

Key Points • Search for other sites of disease, including the brain and pituitary gland, heart, lungs, and bones.

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Fig. 4.32  A 61-year-old male with Erdheim-Chester disease who presented with orbital pain and blurry vision. (a) Axial T2 MRI without fat saturation shows hypointense intraconal disease in bilateral orbits (arrows). (b) Axial T1 post-contrast MRI with fat saturation shows heterogeneous enhancement of the intraconal disease (arrows) with

involvement of the left cavernous sinus (thin arrow). (c) Coronal T1 post-contrast MRI with fat saturation shows enhancing bilateral intraconal and extraconal disease (arrows), and enhancing disease in the maxillary sinuses (thin arrows)

Infection

including proptosis, vision loss, and painful ophthalmoplegia. • A subperiosteal abscess appears as a peripherally enhancing fluid collection with a lenticular shape along the orbital wall that can laterally displace the medial rectus muscle [142].

Figure 4.33 shows cases of orbital infection.

Background

• Orbital infections account for greater than half of all primary orbital abnormalities. CT • Approximately two-thirds related to sinusitis and one-­ fourth result from intraorbital foreign bodies [142]. • An orbital abscess appears as a peripherally enhancing fluid collection in the orbit. Adjacent soft-tissue standing and EOM involvement [142].

Presentation

• Orbital abscess is a serious complication of orbital cellulitis. • A subperiosteal abscess may develop from acute sinusitis involving the ethmoid air cells [143].

Imaging • Preseptal cellulitis characterized by swelling and stranding of the periorbital soft tissues anterior to the orbital septum [144]. • Orbital cellulitis demonstrates stranding of the soft tissues posterior to the orbital septum with clinical signs

MRI • An orbital abscess appears as a peripherally enhancing fluid collection with restricted diffusion centrally on DWI [142].

Key Points • Search for preseptal versus postseptal involvement. • Describe the presence, size, and location of an abscess. • Assess for signs of sinusitis.

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Fig. 4.33 (a, b) A 64-year-old female with COVID-19 infection, invasive fungal sinusitis, and a right orbital abscess. (a) Axial CT with contrast, soft tissue window shows a peripherally enhancing abscess in the medial right orbit (arrow). (b) Coronal CT with contrast, soft tissue window shows the abscess with lateral displacement of the medial rectus muscle (arrow) and infection in the adjacent ethmoid

air cells (thin arrow). (c) An 81-year-old male with aplastic anemia and left maxillary fungal sinusitis. (c) Coronal CT without contrast, soft tissue window shows inflammatory disease in the left maxillary sinus (arrow) with invasion of the left orbit and enlargement of the medial and inferior rectus muscles (thin arrows)

Venous Malformation

Imaging

Figures 4.34 and 4.35 show cases of orbital venous malformations.

• Well-circumscribed, rounded intraconal lesions. • Due to a slow-flow arterial supply, contrast does not fill the lesion entirely until the late venous phase. • Bone remodeling and intralesional microcalcifications may occur [145].

Background • Venous malformations (cavernous hemangiomas) represent the most common vascular lesion in adults. • These lesions have a fibrous capsule surrounding endothelial-­lined spaces and demonstrate slow progressive enlargement over time 145]. • Do not involute [145]. • Slow flow vascular malformation [4].

Presentation

CT • Hyperdense on non-contrast CT. • May displace adjacent structures without invasion. • Contrast does not completely fill the lesion until the late venous phase [4, 145]. • Phleboliths may be present [4].

MRI

• More common in women, often detected in the second to fourth decades [4]. • Usually solitary and most often arise in the intraconal space [145]. • Often an incidental finding during imaging for clinical symptoms such as headache, pain, proptosis, diplopia, palpable mass, and vision changes [146].

• T1 isointense and T2 hyperintense. • In larger lesions, internal septa may be visualized. • Contrast does not completely fill the lesion entirely until the late venous phase [145].

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Fig. 4.34  A 72-year-old male with treated retroperitoneal liposarcoma who underwent MRI for vertigo, which showed an incidental left orbital venous malformation. (a) Axial T2 MRI with fat saturation shows a hyperintense lesion in the left orbital apex (arrows). (b–d) Axial,

coronal, and sagittal T1 post-contrast MRI with fat saturation shows gradual filling of the venous malformation with contrast on sequential series (arrows)

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Fig. 4.35  A 46-year-old male who underwent MRI for headaches which showed a left orbital venous malformation. (a) Axial T2 MRI with fat saturation shows a hyperintense lesion in the intraconal

space of the left orbit (arrows). (b, c) Axial and sagittal T1 post-­ contrast MRI with fat saturation show gradual filling of the venous malformation with contrast on sequential series (arrows)

Key Points • Important to distinguish between venous, venolymphatic, and arteriovenous malformations, and varices. Different management strategies are appropriate, depending on the type of vascular lesion. • Phleboliths can appear as signal voids and lead to the misdiagnosis of this low-flow lesion as a high-flow lesion. • T2 hyperintense with delayed filling of contrast.

• Females and males are affected with an equal frequency [149]. • A VLM within the orbit may enlarge over time leading to progressive proptosis, eye movement restriction, or globe displacement. • May present abruptly from hemorrhage that may occur following minor trauma, or infection or develop spontaneously, leading to acute proptosis and occasionally optic nerve compression [150].

Venolymphatic Malformation

Imaging

Figure 4.36 shows a case of an orbital venolymphatic malformation.

• Fluid-fluid levels within multiple cysts arising from hemorrhage of various ages is almost pathognomonic of a VLM. • Orbital VLMs are isolated from the normal orbital vasculature and unaffected by postural changes, differentiating them from varices [149]. • Unencapsulated and therefore can be multicompartmental, often involving both the intraconal and extraconal spaces [149].

Background • A venolymphatic malformation (VLM) is a slow-flow vascular malformation arising from the pluripotent venous anlage that develops into both venous and lymphatic structures. • Consists of endothelial-lined lymph-filled vascular channels with variable luminal diameters [147].

Presentation • VLMs may be evident at birth but usually manifest in infancy or childhood [148].

CT • VLMs are multicompartmental, not well-circumscribed. Lymphatic components demonstrate minimal enhancement. Venous component enhances. • Phleboliths can be present [151].

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4 Orbit Fig. 4.36  A 19-year-old male with increasing proptosis related to a left orbital venolymphatic malformation. (a) Axial T2 MRI with fat saturation shows a lobulated mass in the superior left orbit with multiple cysts containing fluid-fluid levels (arrows). (b) Axial T1 post-contrast MRI with fat saturation shows multicompartment involvement and cysts with fluid-fluid levels (arrows)

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Presentation

• Fluid-fluid levels within cysts related to hemorrhages of various ages [149]. • T1 images demonstrate lymphatic and proteinaceous fluid. • T2 fat-suppressed images best show non-hemorrhagic fluid [146].

• Presents in the second or third decade with no gender bias [152]. • Most varices communicate with the venous system [150].

Key Points • Phleboliths can appear as flow voids, leading to the misdiagnosis of a high-flow lesion. • Important to distinguish between venous, venolymphatic, and arteriovenous malformations, and varices. Different management strategies are appropriate depending on the type of vascular lesion. • VLMs can be treated with intralesional sclerotherapy. • Comment on the need for additional studies such as CT/MR angiography for better characterization of the lesion.

Varix

Imaging • Smooth contours may appear club-like, triangular, segmentally dilated, or as a tangled vessel mass. • Maneuvers that increase venous pressure (e.g., scanning with prone positioning or during the Valsalva maneuver) demonstrate distension of varix [150, 153].

CT • Involved veins have a normal appearance or mild enlargement.

MRI

Figures 4.37 shows a case of an orbital varix.

• T1 hypo- to hyperintense and T2 hyperintense with intense enhancement [150].

Background

Key Points

• Varices result from a presumed congenital weakness of the post-capillary venous wall, leading to the proliferation of venous components and marked dilation of the orbital veins [152].

• Important to distinguish between venous, venolymphatic, and arteriovenous malformations, and varices. Different management strategies are appropriate depending on type of vascular lesion.

158 Fig. 4.37  A 61-year-old male with anaplastic thyroid carcinoma and incidentally discovered bilateral orbital varices. (a) Axial CT with contrast, soft tissue window shows homogeneously enhancing lesions in the posterior orbits (arrows). (b) Axial CT with contrast, soft tissue window while performing the Valsalva maneuver shows enlargement of the varices (arrows)

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• Valsalva maneuvers may demonstrate distension of varix.

Imaging • Rapid filling of an AVM with contrast reaching the venous components during the arterial phase [159].

Arteriovenous Malformation Figure 4.38 shows a case of an arteriovenous malformation involving the orbit.

CT • Feeding arteries and draining veins can be visualized especially with CT angiography [158].

Background • Arteriovenous malformations (AVMs) feature a nidus at the confluence of feeding arteries and draining veins without intervening capillaries [154, 155]. • Present at birth in a quiescent stage and do not present clinically until childhood or adulthood [156]. • Growth of the AVM may be exacerbated by hormonal changes of puberty or pregnancy, or result from trauma, thrombosis, or infection [157].

Presentation

MRI • Serpiginous dilated vessels with feeding arteries and draining veins. • Lack a well-defined mass and appear as flow voids, indicating the high-flow components [156]. • Associated T1 signal hyperintensity may represent hemorrhage, intravascular thrombus, or flow-related enhancement [160].

Key Points

• AVMs are high-flow vascular lesions. • Symptoms such as pain, bleeding, and overgrowth depend on the degree of arteriovenous shunting [155]. • Facial AVMs involving the skin or facial bones may cause facial asymmetry, bleeding, or skin and mucosal ulcerations that can become secondarily infected [158].

• High-flow vascular lesion. • Important to distinguish between venous, venolymphatic, and arteriovenous malformations, and varices. Different management strategies are appropriate depending on type of vascular lesion. • AVM may benefit from pre-operative embolization.

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Fig. 4.38  A 28-year-old male with a congenital arteriovenous malformation and vision loss in the left eye. (a) Axial CT with contrast, soft tissue window shows multiple dilated enhancing vessels about the face (arrows). (b) Volume-rendered CT with contrast shows serpigi-

nous dilated vessels about the face and orbit. (c, d) Axial T1 post-­ contrast MRI with fat saturation shows the hypointense appearance of the vessels consistent with high flow vascular components (arrows)

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• Comment on the need for additional studies such as CT/MR angiography for better characterization of the lesion.

Imaging

Superior Ophthalmic Vein Thrombosis

• Superior ophthalmic vein appears dilated and thrombotic. • There may be evidence of sinusitis or cellulitis or a history of functional sinus surgery.

Figure 4.39 shows cases of superior ophthalmic vein thrombosis.

CT/MRI

Background • Superior ophthalmic vein thrombosis is extremely rare with potentially devastating consequences [161, 162]. • Etiologies: orbital cellulitis, paranasal sinusitis, trauma, cavernous sinus thrombosis, cavernous sinus thrombosis fistula, orbital neoplasm, hypercoagulable states, and Tolosa–Hunt syndrome [161].

Presentation • Symptoms: periorbital edema, proptosis, globe dystopia, ophthalmoplegia, ptosis, proptosis, and chemosis (conjunctival edema) [161]. Fig. 4.39  A 71-year-old male with right orbital swelling, vision loss, and thrombocytosis (elevated platelet count). (a, b) Axial and coronal CT with contrast, soft tissue window shows contrast filling the left superior ophthalmic vein (white arrows) and lack of enhancement in the dilated right superior ophthalmic vein (black arrows). Note proptosis of the right globe and enlargement of the right extra-ocular muscles

• Linear filling defect in a dilated superior ophthalmic vein related to the thrombosis on CT and MR venography. • Superior ophthalmic vein and/or cavernous sinus thrombosis may not be evident in the early stages. • Some authors believe that MRI is more sensitive for diagnosis [163].

Key Points • Search for imaging signs of the potential causes of superior ophthalmic vein thrombosis, e.g., orbital cellulitis, sinusitis, cavernous sinus thrombosis/fistula, or an orbital lesion.

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4 Orbit 138. Arnaud L, Hervier B, Néel A, Hamidou MA, Kahn JE, Wechsler B, et al. CNS involvement and treatment with interferon-α are independent prognostic factors in Erdheim-Chester disease: a multicenter survival analysis of 53 patients. Blood. 2011;117:2778–82. https://doi.org/10.1182/blood-­2010-­06-­294108. 139. Drier A, Haroche J, Savatovsky J, Godenèche G, Dormont D, Chiras J, et al. Cerebral, facial, and orbital involvement in Erdheim-­ Chester disease: CT and MR imaging findings. Radiology. 2010;255:586–94. https://doi.org/10.1148/radiol.10090320. 140. Sedrak P, Ketonen L, Hou P, Guha-Thakurta N, Williams MD, Kurzrock R, et  al. Erdheim-Chester disease of the central nervous system: new manifestations of a rare disease. AJNR Am J Neuroradiol. 2011;32:2126–31. https://doi.org/10.3174/ajnr. A2707. 141. Mamlouk MD, Aboian MS, Glastonbury CM. Case 245: Erdheim-­ Chester disease. Radiology. 2017;284:910–7. https://doi. org/10.1148/radiol.2017141151. 142. Nguyen VD, Singh AK, Altmeyer WB, Tantiwongkosi B.  Demystifying orbital emergencies: a pictorial review. Radiographics. 2017;37:947–62. https://doi.org/10.1148/ rg.2017160119. 143. Rahbar R, Robson CD, Petersen RA, DiCanzio J, Rosbe KW, McGill TJ, et al. Management of orbital subperiosteal abscess in children. Arch Otolaryngol Head Neck Surg. 2001;127:281–6. https://doi.org/10.1001/archotol.127.3.281. 144. Givner LB.  Periorbital versus orbital cellulitis. Pediatr Infect Dis J. 2002;21:1157–8. https://doi. org/10.1097/00006454-­200212000-­00014. 145. Ansari SA, Mafee MF.  Orbital cavernous hemangioma: role of imaging. Neuroimaging Clin N Am. 2005;15:137–58. https://doi. org/10.1016/j.nic.2005.02.009. 146. Bilaniuk LT.  Vascular lesions of the orbit in children. Neuroimaging Clin N Am. 2005;15:107–20. https://doi. org/10.1016/j.nic.2005.03.001. 147. Katz SE, Rootman J, Vangveeravong S, Graeb D.  Combined venous lymphatic malformations of the orbit (so-called lymphangiomas). Association with noncontiguous intracranial vascular anomalies. Ophthalmology. 1998;105:176–84. https://doi. org/10.1016/s0161-­6420(98)92058-­9. 148. Wright JE, Sullivan TJ, Garner A, Wulc AE, Moseley IF. Orbital venous anomalies. Ophthalmology. 1997;104:905–13. https://doi. org/10.1016/s0161-­6420(97)30208-­5. 149. Harris GJ, Sakol PJ, Bonavolontà G, De Conciliis C. An analysis of thirty cases of orbital lymphangioma. Pathophysiologic considerations and management recommendations. Ophthalmology. 1990;97:1583–92. https://doi.org/10.1016/s0161-­6420(90)32370-­9. 150. Smoker WR, Gentry LR, Yee NK, Reede DL, Nerad JA. Vascular lesions of the orbit: more than meets the eye. Radiographics. 2008;28:185–204. https://doi.org/10.1148/rg.281075040.

165 151. Graeb DA, Rootman J, Robertson WD, Lapointe JS, Nugent RA, Hay EJ. Orbital lymphangiomas: clinical, radiologic, and pathologic characteristics. Radiology. 1990;175:417–21. https://doi. org/10.1148/radiology.175.2.2326469. 152. Rubin PA, Remulla HD. Orbital venous anomalies demonstrated by spiral computed tomography. Ophthalmology. 1997;104:1463– 70. https://doi.org/10.1016/s0161-­6420(97)30115-­8. 153. Winter J, Centeno RS, Bentson JR. Maneuver to aid diagnosis of orbital varix by computed tomography. AJNR Am J Neuroradiol. 1982;3:39–40. 154. Bigot JL, Iacona C, Lepreux A, Dhellemmes P, Motte J, Gomes H.  Sinus pericranii: advantages of MR imaging. Pediatr Radiol. 2000;30:710–2. https://doi.org/10.1007/s002470000306. 155. Flors L, Leiva-Salinas C, Maged IM, Norton PT, Matsumoto AH, Angle JF, et al. MR imaging of soft-tissue vascular malformations: diagnosis, classification, and therapy follow-up. Radiographics. 2011;31:1321–40; discussion 1340–1. https://doi.org/10.1148/ rg.315105213. 156. Ernemann U, Kramer U, Miller S, Bisdas S, Rebmann H, Breuninger H, et al. Current concepts in the classification, diagnosis and treatment of vascular anomalies. Eur J Radiol. 2010;75:2– 11. https://doi.org/10.1016/j.ejrad.2010.04.009. 157. Donnelly LF, Adams DM, Bisset GS 3rd. Vascular malformations and hemangiomas: a practical approach in a multidisciplinary clinic. AJR Am J Roentgenol. 2000;174:597–608. https://doi. org/10.2214/ajr.174.3.1740597. 158. McCafferty IJ, Jones RG.  Imaging and management of vascular malformations. Clin Radiol. 2011;66:1208–18. https://doi. org/10.1016/j.crad.2011.06.014. 159. Nosher JL, Murillo PG, Liszewski M, Gendel V, Gribbin CE. Vascular anomalies: a pictorial review of nomenclature, diagnosis and treatment. World J Radiol. 2014;6:677–92. https://doi. org/10.4329/wjr.v6.i9.677. 160. Abernethy LJ. Classification and imaging of vascular malformations in children. Eur Radiol. 2003;13(11):2483–97. https://doi. org/10.1007/s00330-­002-­1773-­8. 161. Park HS, Gye HJ, Kim JM, Lee YJ. A patient with branch retinal vein occlusion accompanied by superior ophthalmic vein thrombosis due to severe superior ophthalmic vein enlargement in a patient with graves ophthalmopathy. J Craniofac Surg. 2014;25:e322–4. https://doi.org/10.1097/SCS.0000000000000586. 162. Gupta RK, Jamjoom AA, Devkota UP.  Superior sagittal sinus thrombosis presenting as a continuous headache: a case report and review of the literature. Cases J. 2009;2:9361. https://doi. org/10.1186/1757-­1626-­2-­9361. 163. Valera FC, dos Santos AC, Anselmo-Lima WT, Marquezini RM.  Orbital complications of acute rhinosinusitis: a new classification. Braz J Otorhinolaryngol. 2007;73:684–8. https://doi. org/10.1016/s1808-­8694(15)30130-­0.

5

Skull Base and Bone J. Matthew Debnam, Franco Rubino, and Shaan M. Raza

Tumors and non-neoplastic lesions involve the bones and adjacent soft tissues of the maxillofacial region and skull base. These occur in children and adults and include a wide range of benign and malignant lesions. Benign lesions include meningiomas and aneurysmal bone cysts. Malignant tumors include sarcomas such as osteosarcomas, chondrosarcomas, and fibrosarcoma and other tumors such as nasopharyngeal carcinoma and metastases. Other lesions that mimic tumors include developmental cysts, fibrous dysplasia, and osteomas. Not only may these lesions invade the orbit, but they can also involve the cavernous sinus, sella turcica, and cranial nerves. Knowledge of the imaging features of these tumors is essential for patient care, including surgical resection and avoiding unnecessary treatments. The imaging modalities used to evaluate the skull base include CT, MRI, and PET/CT.  These modalities provide important information about staging, pre-surgical planning, and treatment response. CT aids in the delineation of tumor extent and bone remodeling or destruction. MRI also evaluates the features of the tumors, including soft tissue characteristics, sinonasal and intracranial involvement, and perineural tumor spread. PET/CT provides information for assessing a tumor’s metabolic activity, detecting local and distant metastases, staging, determining a site for biopsy based on metabolic activity, and evaluating treatment response. The purpose of this chapter is to describe the demographics and imaging appearance of common and uncommon malignancies and conditions of the bones and adjacent soft tissues of the maxillofacial region and skull base. This is J. M. Debnam (*) Department of Neuroradiology, Division of Diagnostic Imaging, The University of Texas MD Anderson Cancer Center, Houston, TX, USA e-mail: [email protected] F. Rubino · S. M. Raza Department of Neurosurgery, Division of Surgery, The University of Texas MD Anderson Cancer Center, Houston, TX, USA e-mail: [email protected]; [email protected]

accomplished by reviewing the disease background, clinical presentation, and imaging features on various modalities. The information provided in this chapter should aid the radiologist in narrowing their differential diagnosis when evaluating malignancies of the maxillofacial region and skull base. Tumors arising from the orbit, sinonasal cavity, pituitary gland, cavernous sinus, and brain are discussed in other chapters.

Anatomy Figures 5.1 and 5.2 show schematic, CT and MRI anatomy of the skull base.

Anterior Skull Base • Separates the intracranial compartment from the orbits and paranasal sinuses. • Includes the area of the posterior bony wall of the frontal sinus to the lesser wings of the sphenoid bone and the anterior clinoid processes. • Bounded laterally by the orbital plates of the frontal bone. • Includes the cribriform plate and planum sphenoidale.

Central Skull Base • Includes the area posterior to the lesser wings of the sphenoid bone and the planum sphenoidale to the temporal bones and petrous ridges. • Incorporates the middle cranial fossae laterally. • Central component contains the sella turcica and ventral portion of the clivus. • Spheno-occipital and petroclival synchondroses mark the posterior border medially and laterally, respectively.

© The Author(s), under exclusive license to Springer Nature Switzerland AG 2023 J. M. Debnam (ed.), Imaging Atlas of Ophthalmic Tumors and Diseases, https://doi.org/10.1007/978-3-031-17479-7_5

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Lamina cribrosa (I) Optic canal (II)

Orbital roof

Frontal bone Cribriform plate of ethmoid bone

Superior orbital fissure (CN III, IV, V1, V); orbital branch (middle meningeal artery), superior ophtalmic vein)

Anterior cranial fossa

Foramen rotundum (V2)

Lesser wing of the sphenoid

Anterior cranial fossa Optic canal

Carotid canal Foramen ovale (V3) Sella turcica

Middle cranial fossa

Temporal bone (pars petrosa)

Clivus

Foramen spinosum (middle meningeal artery & vein, meningeal branch [V3]) Foramen lacerum & sphenopetrosal fissure (Greater petrosal nerve [VII], lesser petrosal nerve [IX], deep petrosal nerve [carotid plexus])

Foramen rotundum

Hypophyseal fossa

Foramen ovale

Parietal bone

Jugular foramen

Internal acoustic meatus (CN VII, VIII) Jugular foramen (CN IX, X, XI; internal jugular vein) Posterior cranial fossa

Foramen magnum (medulla oblongata, spinal route of CN IX, anterior spinal artery, vertebral artery, intraspinal venous plexus)

Posterior cranial fossa Foramen magnum Occipital bone Bones of the skull base

Fig. 5.1  Schematic drawings of the skull base. (a reprinted from Borghei-Razavi et al. [1]; with permission; b reprinted from Audu et al. [2]; with permission)

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Fig. 5.2  Skull base foramina anatomy. (a) Axial CT with contrast, bone window shows the superior orbital fissures (large white arrows) and the optic canals (thin white arrows). (b) Axial CT with contrast, bone window shows pterygopalatine fossae (large white arrow), foramen ovale (large black arrows), the petroclival fissures (thin white

arrows), and clivus (thin black arrow). (c) Coronal CT with contrast, bone window shows the foramen rotundum (large white arrows) and carotid canals (thin white arrows). (d) Coronal CT with contrast, bone window shows the foramen ovale (white arrows)

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Figures 5.3, 5.4, and 5.5 show examples of meningiomas.

• In grade I lesions, a cleft of cerebrospinal fluid (CSF) is present between the meningioma and brain parenchyma. • Grade III lesions are aggressive with invasion of the surrounding structures. –– Loss of the CSF cleft without delineation between the meningioma and adjacent brain parenchyma may be present [5]. • Hyperostosis of adjacent bone in 20% and calcification in 25% [5]. • Cystic changes and necrosis may be present. • Internal carotid artery encasement can cause vessel stenosis. • Pneumosinus dilatans (dilation of an adjacent sinus) [9]. • Growth through skull base and orbital foramina can result in cranial nerve palsies [5].

Background

CT

• Most often originate from arachnoid cap cells or meningocytes of the meninges. • Usually benign lesion; represents the most common tumor of the dura. • The World Health Organization classification is based on the histological characteristics and the risk of recurrence. –– Grade I (benign [80%]), grade II (atypical [18%]), and grade III (malignant [2%]) [3, 4]. • Most sporadic. Some may be familial or occur following radiation therapy. • Neurofibromatosis type 2 is associated with multiple meningiomas [5]. • In rare instances, a primary intraosseous meningioma (PIM) arises within the bone [6].

• Hyperdense (60%) to isodense (40%) to cortex [10]. • Intraosseous meningiomas may show intra- and extraosseous involvement and destruction of the cortex [11].

Presentation

•

• Contains the optic canal, superior orbital fissure, internal carotid artery, cavernous sinus, foramen rotundum, foramen ovale and foramen spinosum, and the Vidian canal.

Posterior Skull Base • Extends centrally from the spheno-occipital synchondrosis to the inner table of the occipital bone. • Extends posterolaterally from the petrous ridge to the inner tables of the temporal and occipital bones.

Meningioma

• Predominantly occurring in females; peak incidence in the fifth to sixth decades. • Symptoms depend on location: headache, loss of vision, and nerve palsies [5, 7]. Visual field defects occur with compression upon the optic chiasm [8]. When decussating optic nerve fibers are affected, there may be loss of stereopsis (depth perception) [6].

Imaging • Strong uniform enhancement and a dural tail (thickening and enhancement of the dural). –– not specific for a meningioma.

MRI • Usually isointense to the cortex on all sequences. • Shows extradural extension and soft tissue involvement. • MR spectroscopy (MRS), high choline and alanine peaks with depression of the N-acetyl aspartate (NAA) peak [7].

PET Ga-DOTATATE involvement [12]. 68

PET/CT

demonstrates

osseous

Surgical Key Points • Anterior and middle fossa meningiomas can extend intracranially (affecting neurovascular structures) and extracranially (affecting the orbits, nasal sinuses, and infratemporal fossa). • For intraosseous meningiomas, obtain a CT scan to determine bone erosion. • Describe extraconal or intraconal space extension when dealing with orbital extension. • Describe cavernous sinus wall patency and tumor extension through superior or inferior orbital fissures.

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Fig. 5.3  A 57-year-old female with acute vision loss in the right eye secondary to a sphenoid wing meningioma. (a) Axial CT with contrast, soft tissue window shows an enhancing meningioma involving the right sphenoid bone with extension into the lateral extraconal space of the right orbit, middle cranial fossa, and masticator space (arrows). (b) Axial CT with contrast, bone window shows hyperostosis

of the right sphenoid bone and lateral orbital wall (arrows). (c) Axial T2 MRI with fat saturation shows a hypointense appearance of the hyperostotic bone (arrow) and an isointense appearance of the meningioma (thin arrows). (d) Axial T1 post-contrast MRI with fat saturation shows enhancement of the meningioma (arrows)

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Fig. 5.4  A 59-year-old male with 3 months of progressive bulging of the left eye associated with increasing blurry vision due to a sphenoid wing meningioma. (a) Axial CT without contrast, bone window shows irregularity of the left sphenoid bone (arrow). (b) Axial T1 non-­ contrast MRI without fat saturation shows an isointense appearance of the meningioma in the left posterolateral orbit, middle cranial

fossa, and masticator space (arrows). (c) Axial T2 MRI with fat saturation shows an isointense appearance of the meningioma (arrows). Note the CSF cleft along the posterior margin (thin arrow). (d) Axial T1 post-contrast MRI with fat saturation demonstrating homogeneous enhancement of the meningioma (arrows)

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Fig. 5.5  A 56-year-old female presented with a complaint of dizziness. (a) Axial CT with contrast, soft tissue window shows a hyperdense meningioma in the region of the left cavernous sinus, left middle cranial fossa, and posterior cranial fossa with mass effect upon the pons (arrows). (b) Axial CT with contrast, bone window shows associated hyperostosis of the left posterior clinoid process (arrow). (c)

Axial T2 MRI with fat saturation shows an isointense to slightly hyperintense appearance of the meningioma (arrow) and encasement of the basilar artery flow void (thin arrow). (d) Axial T1 post-­ contrast MRI with fat saturation shows homogeneous enhancement of the meningioma (arrows)

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• Describing the tumor’s possible origin helps determine the best surgical approach. –– Midline meningiomas, e.g., olfactory groove and planum or tuberculum sellae meningiomas, could be treated with endoscopic endonasal approaches. –– More lateral meningiomas, e.g., clinoid or medial sphenoid wing meningiomas are treated with a transcranial approach. • For perioptic meningiomas, describe optic apparatus compression (which segment of the apparatus is involved), sellar involvement, and extension to the sphenoid sinus.

Osteosarcoma Figures 5.6, 5.7, and 5.8 show examples of osteosarcomas.

Background • Osteosarcoma (OSA) is the most common primary malignant bone tumor. • Most occur in the metaphysis of the long bones. • Head and neck OSA is rare, accounting for less than 10% of OSA, most commonly in the jaw. • Orbital involvement may be primary or from direct extension [13].

Imaging • Osteoblastic and/or osteolytic lesion with irregular margins. • Characteristic features; osteoid matrix and “sunburst” periosteal reaction [13].

CT • Bone destruction and a soft tissue mass are often present. • Bony sclerosis in approximately 75% of patients. • In some cases, bone destruction without periosteal reaction.

MRI • Signal characteristics depend upon the tumor’s mineral content. –– Often indistinguishable from other sarcomas. • T1 hypointense and heterogeneously T2 iso- to hyperintense with heterogeneous enhancement. • Solid component usually enhances [16]. • Restricted diffusion in up to 30% due to dense cellularity [9].

PET

Presentation

• • Primary OSA typically in younger patients (