Craniopharyngiomas - Classification and Surgical Treatment [1 ed.] 9781681085326, 9781681085333

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Frontiers in Neurosurgery (Volume 4) (Craniopharyngiomas -Classification and Surgical Treatment) Editor Songtao Qi

Department of Neurosurgery,Nanfang Neurosurgery Research Institution, Nanfang Hospital, Southern Medical University,1838 North Guangzhou Avenue, 510515 Guangzhou,China

Associate Editors Jun Pan and Yuntao Lu

Department of Neurosurgery,Nanfang Neurosurgery Research Institution, Nanfang Hospital, Southern Medical University,1838 North Guangzhou Avenue, 510515 Guangzhou,China

 

Frontiers in Neurosurgery Volume # 4 Craniopharyngiomas - Classification and Surgical Treatment Editor: Songtao Qi Associate Editors: Jun Pan and Yuntao Lu ISSN (Online): 2405-741X ISSN (Print): 2405-7401 ISBN (Online): 978-1-68108-532-6 ISBN (Print): 978-1-68108-533-3 ©2017, Bentham eBooks imprint. Published by Bentham Science Publishers – Sharjah, UAE. All Rights Reserved.

 

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CONTENTS PREFACE ................................................................................................................................................ i LIST OF CONTRIBUTORS .................................................................................................................. iii CHAPTER 1 HISTORY AND EPIDEMIOLOGY OF CRANIOPHARYNGIOMA ..................... Yuntao Lu HISTORY AND TERMINOLOGY .............................................................................................. EPIDEMIOLOGY AND DEMOGRAPHY ................................................................................. CLINICAL MANIFESTATION ................................................................................................... CONFLICT OF INTEREST ......................................................................................................... ACKNOWLEDGEMENTS ........................................................................................................... REFERENCES ...............................................................................................................................

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CHAPTER 2 THE PITUITARY GLAND AND ETIOLOGY OF CRANIOPHARYNGIOMA Yuntao Lu INTRODUCTION .......................................................................................................................... MORPHOLOGY AND CYTOLOGY OF THE PITUITARY GLAND ................................... Anatomical Conceptions ......................................................................................................... Cytology of the Pituitary Gland .............................................................................................. Adenohypophysis Fig. (2) .............................................................................................. Pars Tuberalis .......................................................................................................................... Pars Intermedia ....................................................................................................................... Neurohypophysis .................................................................................................................... Pathological Influence of Pituitary Stalk Dissection .............................................................. Pharyngeal Hypophysis .......................................................................................................... EMBRYONIC DEVELOPMENT OF THE PITUITARY GLAND AND ITS ACCESSORY STRUCTURES ............................................................................................................................... Embryonic Development: Pituitary Gland .............................................................................. Embryonic Development: Pituitary Capsule .......................................................................... ORIGIN OF CRANIOPHARYNGIOMA .................................................................................... Residual Cells of Craniopharyngeal Duct [4, 22, 31, 32, 37] ................................................. Residual Rathke’s Pouch Cells ............................................................................................... Cellular Metaplastic Theory ................................................................................................... CONFLICT OF INTEREST ......................................................................................................... ACKNOWLEDGEMENTS ........................................................................................................... REFERENCES ...............................................................................................................................

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CHAPTER 3 PATHOLOGICAL CLASSIFICATION OF CRANIOPHARYNGIOMA AND ITS MOLECULAR ASPECTS ...................................................................................................................... Xiao-rong Yan and Yi Liu PATHOLOGICAL TYPES ........................................................................................................... IMAGING FEATURES OF CRANIOPHARYNGIOMA .......................................................... DIFFERENTIAL DIAGNOSIS ..................................................................................................... HARD STRUCTURES IN CRANIOPHARYNGIOMA (CALCIFICATION, OSSIFICATION AND DENTAL TISSUE) ................................................................................. CONFLICT OF INTEREST ......................................................................................................... ACKNOWLEDGEMENTS ........................................................................................................... REFERENCES ...............................................................................................................................

1 2 6 10 10 10

15 16 16 18 18 20 21 22 24 25 25 25 27 29 29 31 33 34 34 34 38 39 42 42 44 49 49 49

CHAPTER 4 CURRENT THERAPEUTIC SITUATION ................................................................ 52 Yi Liu MANAGEMENT DURING THE PERIOPERATIVE PERIOD ............................................... Preoperative Evaluation .......................................................................................................... Preoperative Management ...................................................................................................... Hydrocephalus and Increased Intracranial Pressure ................................................... Hormone Replacement Therapy .................................................................................... Postoperative Management ..................................................................................................... Anti-Epileptic ................................................................................................................ Hormone Replacement Therapy .................................................................................... Electrolyte Disturbances ............................................................................................... Diabetes Insipidus ......................................................................................................... Sudden Disturbance in Consciousness .......................................................................... Other Complications ..................................................................................................... SURGICAL TREATMENT FOR CRANIOPHARYNGIOMA ................................................ Transcranial Approach ............................................................................................................ Transsphenoidal Approach ..................................................................................................... Prognostic Factors for Tumor Recurrence .............................................................................. Long-term Outcomes of Craniopharyngioma After Resection ............................................... RADIOTHERAPY FOR CRANIOPHARYNGIOMA ............................................................... External Beam Irradiation ....................................................................................................... Stereotactic Radiotherapy ....................................................................................................... Proton Beam Therapy ............................................................................................................. Internal Irradiation (Brachytherapy) ....................................................................................... Stereotactic Radiosurgery ....................................................................................................... Radiation-induced Disturbances in Hypothalamic Pituitary Function ................................... CHEMOTHERAPY FOR CRANIOPHARYNGIOMA ............................................................. Intracavitary Chemotherapy .................................................................................................... Systemic Chemotherapy ......................................................................................................... CONFLICT OF INTEREST ......................................................................................................... ACKNOWLEDGEMENT ............................................................................................................. REFERENCES ............................................................................................................................... CHAPTER 5 EXPERIMENTAL CRANIOPHARYNGIOMA RESEARCH ................................. Guang-long Huang and Jie Zhou INTRODUCTION .......................................................................................................................... CELL CULTURE AND TUMORIGENICITY IN VIVO ......................................................... Cell Culture in Vitro .............................................................................................................. Tumorigenicity In Vivo ......................................................................................................... CRANIOPHARYNGIOMA AND INFLAMMATION ............................................................... CRANIOPHARYNGIOMA AND THE EPITHELIAL-MESENCHYMAL TRANSITION (EMT) ............................................................................................................................................... CONFLICT OF INTEREST ......................................................................................................... ACKNOWLEDGEMENTS ........................................................................................................... REFERENCES ...............................................................................................................................

52 53 55 55 56 56 56 56 57 57 58 58 58 59 61 62 62 64 64 65 66 66 67 68 68 68 70 73 73 73 88 88 89 89 91 92 93 95 95 95

CHAPTER 6 ANATOMY BASED ON PTERIONAL APPROACH AND ITS EXTENSION APPROACH FOR SURGERY OF CRANIOPHARYNGIOMA ....................................................... 98 Yuntao Lu PTERIONAL APPROACH AND ITS EXTENSION ................................................................. 98 Historical Overview ................................................................................................................ 98

Position and Surgical Technique ............................................................................................ Anatomical Figures (Figs. 1-8) ............................................................................................... PTERIONAL APPROACH WITH SUB-TEMPORAL EXTENSION ..................................... Anatomical Figures (Figs. 9-16) ............................................................................................. SURGERY OF CRANIOPHARYNGIOMA ............................................................................... CONFLICT OF INTEREST ......................................................................................................... ACKNOWLEDGEMENTS ........................................................................................................... REFERENCES ............................................................................................................................... CHAPTER 7 ANATOMY BASED ON INTERHEMISPHERIC APPROACH FOR SURGERY OF CRANIOPHARYNGIOMA ............................................................................................................. Yuntao Lu FRONTOBASAL INTERHEMISPHERIC APPROACH .......................................................... Historical Overview ................................................................................................................ Position and Surgical Technique ............................................................................................ Anatomical Figures (Figs. 1-9) ............................................................................................... TRANSCALLOSAL AND INTERFONIX APPROACH ........................................................... Historical Overview ................................................................................................................ Position and Surgical Technique ............................................................................................ Approach Figures (Figs. 10-15) .............................................................................................. SURGERY FOR CRANIOPHARYNGIOMA ............................................................................. CONFLICT OF INTEREST ......................................................................................................... ACKNOWLEDGEMENTS ........................................................................................................... REFERENCES ............................................................................................................................... CHAPTER 8 ANATOMY BASED ON TRANSSPENOIDAL APPROACHES FOR SURGERY OF CRANIOPHARYNGIOMA ............................................................................................................. Yuntao Lu TRANSNASAL TRANSSHPENOIDAL APPROACH AND EXTENSIONS .......................... Historical Overview ................................................................................................................ Position and Surgical Technique ............................................................................................ Approach Figures (Figs. 1-14) ................................................................................................ SURGERY OF CRANIOPHAYRNGIOMA ............................................................................... CONFLICT OF INTEREST ......................................................................................................... ACKNOWLEDGEMENTS ........................................................................................................... REFERENCES ............................................................................................................................... CHAPTER 9 CRANIOPHARYNGIOMA CLASSIFICATION: HISTORY AND ITS MERIT Yuntao Lu and Jun Pan HITSTORICAL REVIEWS OF CLASSIFICATION ................................................................ GENERAL CLASSIFICATION AND ITS ADVANTAGES AND SHORTCOMINGS ......... ASPS AND FOUR SEGEMENTS OF THE PITUITARY STALK ........................................... TOPOGRAPHIC CLASSIFICATION BASED ON THE RELEVANT PARASELLAR MEMBRANES ................................................................................................................................ ID Pattern ................................................................................................................................ EA Pattern ............................................................................................................................... IA Pattern ................................................................................................................................ SA Pattern ............................................................................................................................... CLINICAL ANALYSIS ................................................................................................................. Infra-Diaphragmatic Tumor .................................................................................................... Suprasellar Extraventricular Tumor ........................................................................................

99 100 102 104 107 113 113 113 116 116 116 117 117 120 120 121 122 124 132 132 132 134 134 134 135 136 141 143 143 143 146 147 149 150 151 152 153 153 154 155 155 156

Extra- and Intra-Ventricular Tumor ........................................................................................ Trans-infundibular Tumor ...................................................................................................... Infundibulo-tuberal Tumor ..................................................................................................... Comparison of Cases Between Adults and Children .............................................................. SURGICAL CLASSIFICATION (“QST” CLASSIFICATION) ............................................... BASIC PRINCIPLES OF OPERATIVE APPROACH SELECTION ...................................... Common Surgical Approach ................................................................................................... Approach Selection Based on Surgical Classification ............................................................ CONFLICT OF INTEREST ......................................................................................................... ACKNOWLEDGEMENTS ........................................................................................................... REFERENCES ...............................................................................................................................

156 157 158 160 162 164 165 172 172 172 172

CHAPTER 10 SUBDIAPHRAGMATIC CRANIOPHARYNGIOMA (TYPE “Q”) ..................... Jun Pan DEFINITION, INCIDENCE AND TUMOR TOPOGRAPHY .................................................. NEUROIMAGING ......................................................................................................................... MORPHOLOGICAL ANALYSIS OF ID-CP ............................................................................. CLINICAL MANIFESTATION OF ID-CP ................................................................................ ENDOCRNINE EVALUATION ................................................................................................... SURGICAL AIMS AND STRATEGY ......................................................................................... SPECIAL CONSIDERATION IN THE MANAGEMENT OF ID-CP ..................................... SELECTION OF SURGICAL APPROACH ............................................................................... SURGICAL TECHNOLOGY AND SKILL ................................................................................ Transsphenoidal Approach ..................................................................................................... Trans-cranial Approach .......................................................................................................... The Relationship Between the Diaphragmatic Sellae and Tumor Fig. (17) ................. PATHOLOGICAL DIAGNOSIS .................................................................................................. Outcomes and Complications ................................................................................................. Surgical Extension ........................................................................................................ Tumor Recurrence ......................................................................................................... Visual Outcome ............................................................................................................. Endocrine Sequelae ....................................................................................................... Postoperative Hypothalamic Status .............................................................................. SUMMARY AND CONCLUSIONS ............................................................................................. CONFLICT OF INTEREST ......................................................................................................... ACKNOWLEDGEMENTS ........................................................................................................... REFERENCES ...............................................................................................................................

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CHAPTER 11 SUBARACHNOID CISTERNAL CRANIOPHARYNGIOMA (TYPE “S”) ........ Jun Pan DEFINITION .................................................................................................................................. ANATOMICAL LOCATION OF THE TUMOR ....................................................................... NEUROIMAGING OF SUBARACHNOID CPS ........................................................................ CLINICAL PRESENTATION OF SUBARACHNOID CPS ..................................................... SURGICAL CONSIDERATION .................................................................................................. SURGICAL DIFFICULTY DEGRADING ................................................................................. SURGICAL APPROACH SELECTION ..................................................................................... SURGICAL TECHNIQUES .......................................................................................................... PROGNOSIS ................................................................................................................................... CONFLICT OF INTEREST ......................................................................................................... ACKNOWLEDGEMENT ............................................................................................................. REFERENCES ...............................................................................................................................

201

176 178 182 185 186 187 188 189 192 192 192 192 194 194 194 194 195 196 196 196 197 197 197

201 203 209 209 210 211 211 212 213 214 214 214

CHAPTER 12 SUPRASELLAR INFUNDIBULO-TUBERAL CRANIOPHARYNGIOMA (TYPE “T”) .............................................................................................................................................. Jun Pan DEFINITION OF “INFUNDIBULO-TUBER CP” ..................................................................... Relationship Between the Tumor and Third Ventricular Floor .............................................. DEMOGRAPHIC INCIDENCE ................................................................................................... CLNICAL MANIFESTATION ..................................................................................................... NEUROIMAGING ......................................................................................................................... SURGICAL TREATMENT .......................................................................................................... Overview ................................................................................................................................. Evaluation of Surgical Risk .................................................................................................... Surgical Approach Selection ................................................................................................... Surgical Techniques ................................................................................................................ Techniques of the Trans-terminalis Approach .............................................................. AcoA Complex ............................................................................................................... PS .................................................................................................................................. EVALUATION OF THE EXTENT OF SURGICAL REMOVAL ........................................... RISK FACTORS FOR RECURRENCE ...................................................................................... PROGNOSIS ................................................................................................................................... Prognostic Evaluation Methods .............................................................................................. Endocrine ................................................................................................................................ Hypothalamus ......................................................................................................................... Visual ...................................................................................................................................... Neuropsychological and Cognitive/Functional Outcome ....................................................... CONFLICT OF INTEREST ......................................................................................................... ACKNOWLEDGEMENTS ........................................................................................................... REFERENCES ............................................................................................................................... CHAPTER 13 INTRAVENTRICULAR CRANIOPHARYNGIOMA? .......................................... Jun Pan and Yuntao Lu INTRAVENTRICULAR CP (IVC) DEFINITION ..................................................................... EMBRYONIC THEORY OF IVC ORIGIN ................................................................................ DEMOGRAPHY, INCIDENCE AND CLINICAL PRESENTATION .................................... NEUROIMAGING ......................................................................................................................... SURGICAL REMOVAL OF IVCS .............................................................................................. Surgical Strategy ..................................................................................................................... Stratification of the 3rd VF and its Relationship with CP ...................................................... Approach Selection ................................................................................................................. CASE PRESENTATION ............................................................................................................... Case No. 1 (Male Patient, 46 Years Old) ................................................................................ Clinical Manifestation ................................................................................................... Physical Examination .................................................................................................... Chemical Examination .................................................................................................. Neuroimaging Study ...................................................................................................... Conventional MRI ......................................................................................................... Surgery .......................................................................................................................... Pathology Study ............................................................................................................ Postoperative Course .................................................................................................... Case No. 2 (Boy, 7 Years Old) ............................................................................................... Clinical Manifestation ................................................................................................... Physical Examination ....................................................................................................

216 217 220 224 224 225 229 229 229 232 233 233 235 236 237 238 240 240 244 245 246 247 248 248 248 254 254 256 256 258 259 259 259 260 261 261 261 262 262 262 262 263 265 265 266 266 267

Chemical Examination .................................................................................................. Neuroimaging Study ...................................................................................................... Surgery .......................................................................................................................... Pathology Study ............................................................................................................ Postoperative Course .................................................................................................... Case No. 3 (Woman, 50 Years Old, Recurrent Case) ............................................................. Clinical Manifestation ................................................................................................... Physical Examination .................................................................................................... Chemical Examination .................................................................................................. Neuroimaging Study ...................................................................................................... Surgery .......................................................................................................................... Pathology Study ............................................................................................................ Postoperative Course .................................................................................................... Case No. 4 (Man, 45 Years Old) ............................................................................................. Clinical Manifestation ................................................................................................... Physical Examination .................................................................................................... Chemical Examination .................................................................................................. Neuroimaging Study ...................................................................................................... Surgery .......................................................................................................................... Pathology Study ............................................................................................................ Postoperative Course .................................................................................................... CONFLICT OF INTEREST ......................................................................................................... ACKNOWLEDGEMENTS ........................................................................................................... REFERENCES ...............................................................................................................................

267 267 267 268 270 270 270 271 271 271 272 273 274 274 274 274 274 275 275 278 278 279 279 279

CHAPTER 14 INFRA-SELLAR AND NASOPHARYNGEAL CRANIOPHARYNGIOMA ....... Jun Pan INTRODUCTION .......................................................................................................................... CLINICAL MANIFESTATION ................................................................................................... DIAGNOSIS AND DIFFERENTIAL DIAGNOSIS .................................................................... SURGICAL TREATMENT .......................................................................................................... CONFLICT OF INTEREST ......................................................................................................... ACKNOWLEDGEMENTS ........................................................................................................... REFERENCES ...............................................................................................................................

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CHAPTER 15 SURGICAL TREATMENT OF RECURRENT CRANIOPHARYNGIOMA ...... Jun Pan INTRODUCTION .......................................................................................................................... RECURRENCE PATTERNS OF CPS ......................................................................................... Recurrence of Subdiaphragmatic CP ...................................................................................... Recurrence of Suprasellar 3rd VF Tumors ............................................................................. CLINICAL CHARACTERISTICS OF RECURRENT CPS ..................................................... THERAPEUTIC STRTEGY FOR RECURRENT CP ............................................................... Surgical Techniques for Recurrent CPs .................................................................................. PROGNOSIS OF RECURRENT CPS ......................................................................................... CONFLICT OF INTEREST ......................................................................................................... ACKNOWLEDGEMENTS ........................................................................................................... REFERENCES ...............................................................................................................................

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283 285 286 286 286 287 287

289 290 291 293 294 294 295 296 298 298 298

CHAPTER 16 HYPOPITUITARISM IN CRANIOPHARYNGIOMA ........................................... 302 Chun-ling Ye and Jun-xiang Peng

LABORATORY EXAMINATION METHODS OF PITUITARY HORMONES ................... Basal Anterior Pituitary Hormone Assays .............................................................................. Dynamic Tests of Anterior Pituitary Function ........................................................................ Assessing the Hypothalamic–Pituitary–Adrenal (HPA) Axis ....................................... Assessing the Hypothalamic–Pituitary–Adrenal (HPA) Axis ....................................... Assessing Growth Hormone Reserve ............................................................................ Assessing the Pituitary–gonadal Axis ........................................................................... Posterior Pituitary Assessment ..................................................................................... CLINICAL MANIFESTATION OF PANHYPOPITUITARISM ............................................. ENDOCRINOLOGICAL EVALUATION BEFORE AND AFTER SURGERY .................... Follow-up ................................................................................................................................ TREATMENT ................................................................................................................................. Hormone Replacement Therapy ............................................................................................. ACTH Deficiency ................................................................................................................... TSH Deficiency ...................................................................................................................... LH/FSH Deficiency ................................................................................................................ Testosterone Cipionate or Testosterone Enanthate Replacement ........................................... Replacement Therapy for LH and FSH Deficiency (women) ................................................ GH Deficiency ........................................................................................................................ Vasopressin Deficiency .......................................................................................................... THE FUTURE ........................................................................................................................ Hypopituitarism Patterns of Adult Craniopharyngioma ......................................................... Endocrinological Assessment ................................................................................................. Endocrine Characteristics Before and After Operation .......................................................... Preoperative Endocrine ................................................................................................ Endocrine Changes in Early Period of Postoperation ............................................................. Endocrine Changes in Mid and Late Postoperative (Tables 2 and 3) ..................................... GROWTH FEATURES OF CHILDHOOD CRANIOPHARYNGIOMAS ............................. CONCLUSION ............................................................................................................................... CONFLICT OF INTEREST ......................................................................................................... ACKNOWLEDGEMENTS ........................................................................................................... REFERENCES ...............................................................................................................................

304 304 304 305 305 305 306 306 307 308 310 311 311 311 312 312 313 313 313 314 314 315 316 317 317 317 317 320 323 323 323 323

CHAPTER 17 HYPOTHALAMUS STATUS EVALUATION ........................................................ Chun-ling Ye and Jun-xiang Peng HYPOTHALAMIC OBESITY IN CRANIOPHARYNGIOMA ............................................... Clinical Characteristics of CP Patients with Hypothalamic Obesity ...................................... Hyperphagia .................................................................................................................. Reduced Physical Activity ............................................................................................. Treatment of Hypothalamic Obesity ....................................................................................... Lifestyle Intervention ..................................................................................................... Pharmacotherapy .......................................................................................................... Surgery .......................................................................................................................... OBESITY AFTER RADICAL RESECTION OF PEDIATRIC CP: RELATIONSHIP WITH TUMOR LOCATION AND GROWTH PATTERN ...................................................... EMOTIONAL, MEMEORY AND CIRCADIAN RHYTHM CHANGES WITH CRANIOPHARYNGIOMA ........................................................................................................... CONFLICT OF INTEREST ......................................................................................................... ACKNOWLEDGEMENTS ........................................................................................................... REFERENCES ...............................................................................................................................

328 328 329 329 329 329 329 330 330 331 335 338 338 338

CHAPTER 18 CLINICAL MANIFESTATION AND MANAGEMENT IN CHILDREN ........... Jun Pan THE CONCEPT OF PEDIATRIC CRANIOPHARYNGIOMA ............................................... PRESENTING MANIFESTATION IN PEDIATRIC CP .......................................................... CLINICAL MANIFESTATION ................................................................................................... NEURORADIOLOGY OF PEDIATRIC CP .............................................................................. THE HEPOTHALAMO-PITUITARY FUNCTION AT PRESENTATION ........................... MORPHOLOGICAL FEATURES OF PEDIATRIC VERSUS ADULT CP .......................... THERAPUTIC STRATEGY FOR PEDIATRIC CP ................................................................. PROGNOSIS OF PEDIATRIC CP .............................................................................................. CONFLICT OF INTEREST ......................................................................................................... ACKNOWLEDGEMENTS ........................................................................................................... REFERENCES ............................................................................................................................... CHAPTER 19 TYPICAL CASES OF CRANIOPHARYNGIOMA ................................................ Jun Pan and Yuntao Lu INFRADIAPHRAGMATIC CRANIOPHARYNGIOMA (TYPE “Q”) ................................... Case 1 ...................................................................................................................................... Medical History ............................................................................................................. Analysis Before Surgery ................................................................................................ Membranous Structures and Hierarchical Layers: (Fig. 2) ......................................... Case 2 ...................................................................................................................................... Medical History ............................................................................................................. Physical and Experimental Examination ...................................................................... Radiological Images Before Surgery ............................................................................ Analysis Before Surgery ................................................................................................ Membranous Structures and Hierarchical Layers: Fig. (5) ......................................... Case 3 ...................................................................................................................................... Medical History ............................................................................................................. Physical and Experimental Examination ...................................................................... Radiological Images Before Surgery ............................................................................ Analysis Before Surgery ................................................................................................ Membranous Structures and Hierarchical Layers: Fig. (8) ......................................... Case 4 ...................................................................................................................................... Medical History ............................................................................................................. Physical and Experimental Examination ...................................................................... Radiological Images Before Surgery ............................................................................ Membranous Structures and Hierarchical Layers: Fig. (11) ....................................... Case 5 ...................................................................................................................................... Medical History ............................................................................................................. Physical and Experimental Examination ...................................................................... Radiological Images Before Surgery ............................................................................ Analysis Before Surgery ................................................................................................ CISTERNAL CRANIOPHARYGIOMA (TYPE “S”) ................................................................ Case 1 ...................................................................................................................................... Medical History ............................................................................................................. Physical and Experimental Examination: Fig. (16) ..................................................... Radiological Images Before Surgery ............................................................................ Analysis Before Surgery ................................................................................................ Membranous Structures and Hierarchical Layers: Fig. (17) ....................................... Case 2 ...................................................................................................................................... Medical History .............................................................................................................

341 341 342 342 343 345 346 350 352 354 354 354 359 359 359 359 360 360 362 362 362 362 363 363 366 366 366 366 366 367 369 369 370 370 370 372 372 372 373 373 375 375 375 375 375 375 376 379 379

Physical and Experimental Examination ...................................................................... Radiological Images Before Surgery ............................................................................ Analysis Before Surgery ................................................................................................ Membranous Structures and Hierarchical Layers: Fig. (21) ....................................... Case 3 ...................................................................................................................................... Medical History ............................................................................................................. Physical and Experimental Examination ...................................................................... Radiological Images Before Surgery ............................................................................ Analysis Before Surgery ................................................................................................ Membranous Structures and Hierarchical Layers: Fig. (24) ....................................... Case 4 ...................................................................................................................................... Medical History ............................................................................................................. Physical and Experimental Examination ...................................................................... Radiological Images Before Surgery ............................................................................ Analysis Before Surgery ................................................................................................ Membranous Structures and Hierarchical Layers ........................................................ Case 5 ...................................................................................................................................... Medical History ............................................................................................................. Physical and Experimental Examination ...................................................................... Radiological Images Before Surgery ............................................................................ Analysis Before Surgery ................................................................................................ CISTERNAL CRANIOPHARYGIOMA (TYPE “S”) ................................................................ Case 1 ...................................................................................................................................... Medical History ............................................................................................................. Physical and Experimental Examination ...................................................................... Radiological Images Before Surgery ............................................................................ Analysis Before Surgery ................................................................................................ Case 2 ...................................................................................................................................... Medical History ............................................................................................................. Physical and Experimental Examination ...................................................................... Radiological Images Before Surgery ............................................................................ Analysis Before Surgery ................................................................................................ Case 3 ...................................................................................................................................... Medical History ............................................................................................................. Physical and Experimental Examination ...................................................................... Radiological Images Before Surgery ............................................................................ Case 4 ...................................................................................................................................... Medical History ............................................................................................................. Physical and Experimental Examination ...................................................................... Radiological Images Before Surgery ............................................................................ Analysis Before Surgery ................................................................................................ Case 5 ...................................................................................................................................... Medical History ............................................................................................................. Physical and Experimental Examination ...................................................................... Radiological Images Before Surgery ............................................................................

379 379 379 380 382 382 382 382 383 384 386 386 386 386 387 387 390 390 390 390 390 393 393 393 393 393 394 397 397 397 397 398 399 399 399 399 402 402 402 403 403 405 405 405 405

CONFLICT OF INTEREST ......................................................................................................... 407 ACKNOWLEDGEMENTS ........................................................................................................... 407 AFTERWORD ......................................................................................................................................... 408 SUBJECT INDEX .................................................................................................................................... 409

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PREFACE In the 30th years of last century, Dr. Harvey Cushing, the great master of neurosurgery, stated that “craniopharyngioma (CP) is the most buffering problem to the neurosurgeon”. Although a century has since passed, this famous comment has been used frequently until now, indicating that the refractoriness of CP continues to challenge both patients and doctors. During the past 10 years, epidemiological data regarding brain tumors have revealed that CP accounts for 6–9% of all intracranial tumors and 54% of pediatric tumors in the sellar region. Furthermore, the annual prevalence of CP within a given population was 1.3/million. The above data confirm that CP is not a rare type of tumor, but rather is a common intracranial tumor in pediatric patients. Histopathologically, CP originates from residual embryonic Rathke’s cyst cells, which are associated with a benign tumor. However, given the extreme difficulty associated with surgical treatment, various treatments for malignant cancers, such as conventional radiotherapy, stereotactic radiosurgery, internal radiation therapy, and chemotherapy, have been accepted by many neurosurgery centers in different countries worldwide. Despite this acceptance, the high rate of recurrence, low quality of life, and low tumor-free survival rate associated with CP unfortunately have not been significantly altered. Taken together, these findings suggest that this benign tumor exhibits malignant biological behaviors. Undoubtedly, surgery is the optimal treatment method for CP. In other words, CP is a strictly surgical disease, and thus surgery might be the only curative method. In light of the advanced development and applications of microsurgical techniques in the most recent half-century, radical surgical CP removal has become increasingly accepted by high-level skull base neurosurgeons, and several publications have reported gross total CP removal rates exceeding 80%. However, given the deep location of CPs in close proximity to vital structures, the high levels of difficulty associated with surgical removal and risk during the perioperative period remain major problems associated with surgical treatment. Regarding reasonable surgical treatment methods for CP, several masters of neurosurgery, including Pascau, Yasargil, and Kassam, summarized large numbers of clinical cases and published several important papers in which they used their experiences to propose methods for selecting the appropriate surgical approach and best intraoperative techniques. These authors also proposed several widely influential clinical classifications of CP, which remain in current usage. Invariably, those classifications accounted for the relationship between the tumor and the third ventricle, and described the anatomical occupation as a purely radiological classification. It is difficult for an inexperienced neurosurgeon to completely understand the true origin of CP, particularly the true relationship of the tumor with the hypothalamic structures. Terminology such as intra-extra-ventricular CP and non-strictly or strictly intra-ventricular CP led to tremendous confusion regarding whether the surgical treatment of CP would inevitably injure the hypothalamic structures. As a result, the concept of unresectable CP was proposed and is considered the primary reason for the wide acceptance of radiotherapeutic and chemotherapeutic treatment of CP. Since 1998, our group has accumulated experience in the surgical treatment of CP, based on the principle of radical gross total removal, in more than 500 cases. First, we paid more attention to the anatomical location of the tumor, and subsequently we shifted our focus to the

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various origins of CP and the relationships between tumors and surrounding vital structures during the development of the various types of CP. Interestingly, the clinical outcome improved dramatically. As a result, we proposed a new classification for CP, according to the stratification of anatomical features and based on the tumor origin.

Major Contributing editors (Left to right): Lu Yuntao, Pan Jun, Qi Songtao, Zhang Xi-an, Fan Jun.

A restored understanding of the anatomical details of CP and their applications resulted in the advanced development of surgical techniques and clinical treatments. Our histological and anatomical studies of the hypophyseal gland, pituitary stalk and third ventricular floor provided a foundation for our clinical applications with regard to sellar region surgery, and comprise a highlighted feature of this book. Of course, a book containing such important clinical and experimental data could not have been completed only by the named authors. We therefore offer our appreciation for all students and observers who have ever studied at our clinical center, all technicians in our laboratory, and the secretaries in my office. An understanding of CP, as well as difficulties of perioperative treatment and long-term hormone substitution, are likely to become strictly required for relevant workers, along with increased patience, thanksgiving and compassion. It is encouraging to note that this attitude has been accepted, inherited, and forwarded by all members of our team. I believe that this quality will not only benefit patients, but will also fill our own lives like a sunny and inspiring breeze. I dedicate this book to all patients and their families for placing their trust in us. In addition, we offer the greatest appreciation to our unsung families for their tremendous and uncomplaining dedication.

Songtao Qi Department of Neurosurgery Nanfang Neurosurgery Research Institution Nanfang Hospital, Southern Medical University 1838 North Guangzhou Avenue 510515 Guangzhou China

iii

List of Contributors Chun-ling Ye

College of Pharmacy, Ji'nan University, 601 Western Huangpu Avenue, Guangzhou, China

Guang-long Huang

Department of Neurosurgery, Nanfang Neurosurgery Research Institution, Nanfang Hospital, Southern medical university, 1838 North Guangzhou avenue, Guangzhou, China

Jie Zhou

Department of Neurosurgery, The First Affiliated Hospital of Sichuan Medical University, 25 Taiping Road, Luzhou, China

Jun Fan

Department of Neurosurgery, Nanfang Neurosurgery Research Institution, Nanfang Hospital, Southern medical university, 1838 North Guangzhou avenue, Guangzhou, China

Jun Pan

Department of Neurosurgery, Nanfang Neurosurgery Research Institution, Nanfang Hospital, Southern medical university, 1838 North Guangzhou avenue, Guangzhou, China

Jun-xiang Peng

Department of Neurosurgery, Nanfang Neurosurgery Research Institution, Nanfang Hospital, Southern medical university, 1838 North Guangzhou avenue, Guangzhou, China

Songtao Qi

Department of Neurosurgery, Nanfang Neurosurgery Research Institution, Nanfang Hospital, Southern medical university, 1838 North Guangzhou avenue, Guangzhou, China

Xi-an Zhang

Department of Neurosurgery, Nanfang Neurosurgery Research Institution, Nanfang Hospital, Southern medical university, 1838 North Guangzhou avenue, Guangzhou, China

Xiao-rong Yan

Department of Neurosurgery, The First Affiliated Hospital of Fujian Medical University, 20 Chazhong Road, Fuzhou, China

Yi Liu

Department of Neurosurgery, Nanfang Neurosurgery Research Institution, Nanfang Hospital, Southern medical university, 1838 North Guangzhou avenue, Guangzhou, China

Yuntao Lu

Department of Neurosurgery, Nanfang Neurosurgery Research Institution, Nanfang Hospital, Southern medical university, 1838 North Guangzhou avenue, Guangzhou, China

Frontiers in Neurosurgery, 2017, Vol. 4, 1-14

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CHAPTER 1

History and Epidemiology of Craniopharyngioma Yuntao Lu* Department of Neurosurgery, Nanfang Neurosurgery Research Institution, Nanfang Hospital, Southern Medical University, 1838 North Guangzhou Avenue, Guangzhou, China Abstract: In this chapter, we firstly briefed reviewed the history and the terminology of craniopharyngioma (CP). Then with summarizing our own data based on 10 top hospitals in China and previous publications, we focused on description of the Epidemiology of craniopharyngioma (CP). And correspondingly, clinical manifestation of CP patients was depicted as well.

Keywords: Craniopharyngioma, Epidemioloty, History, Neurosurgery, Sellar region. HISTORY AND TERMINOLOGY In 1857, Zenker first described a mass composed of squamous epithelial cells along the pars distalis and pars tuberalis of the pituitary [1]. In 1860, Luschka [2] extensively researched adenohypophyseal squamous cells; however, his study was overlooked during the next several decades [3]. Therefore, the origin and terminology of such masses comprising squamous epithelial-like cells remained unclear at that time. In 1902, however, Saxer [4] reported tumors comprising the same type of squamous cells and two years later, after conducting systematic studies of adeno-hypophyseal squamous epithelial cells, Erdheim [5] reported in 1904 that these cells only existed in the pituitary glands of adult patients. Further details regarding location revealed that these cells were usually found on the anterior surface of the infundibulum and were distributed in islets or groups of variable size, shape, and number. Moreover, at that time, several masses comprising these squamous epithelial cells were found to contain various small cysts that were similar to an unnamed type of tumor that originated from the pituitary gland; therefore, the term “hypophyseal duct neoplasms” was used to describe those lesions. Corresponding author Yuntao Lu: Department of Neurosurgery, Nanfang Neurosurgery Research Institution, Nanfang Hospital, Southern Medical University, 1838 North Guangzhou Avenue, Guangzhou, China; Tel/Fax: +862061641806; E-mail: [email protected]

*

Songtao Qi (Ed.) All rights reserved-© 2017 Bentham Science Publishers

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More interestingly, Erdheim found no residual cells along the regressed craniopharyngeal duct, in contrast to a proposal by Von Mihalkovitcs who postulated that during development, the pituitary gland would undergo an upward and forward rotation accompanied by intracranial insertion. It is possible that in terms of the route of development, some residual craniobuccal cyst cells remain. Subsequently, Hirsch O., Duffy, Kiyono, and Carmichael et al. reported similar theories [6]. Along with the development of embryonic pituitary gland studies, several terms were used in later years to describe this type of tumor, including hypophyseal duct tumor, craniopharyngeal duct tumor, Rathke’s pouch tumor, interpeduncular and suprasellar tumors, craniobuccal cysts, suprasellar epithelioma, and adamantinoma. In 1932, Cushing initially proposed the name “craniopharyngioma” to define this type of tumor. Cushing explained this term as follows: “This admittedly somewhat cumbersome term has been employed for want of something more brief to include the kaleidoscopic tumors, solid and cystic, which take their origin from epithelial rests ascribable to an imperfect closure of the hypophyseal or craniopharyngeal duct” [7]. In the same year, Susman observed squamous epithelial cells in the pituitary glands of pediatric patients [8]. EPIDEMIOLOGY AND DEMOGRAPHY Craniopharyngioma, which originates from residual Rathke’s pouch cells, is a type of common benign sellar lesion that accounts for 2-5% of all intracranial tumors [9]. This is also the most common type pituitary mass in children, accounting for 6-13% of all childhood brain tumors and approximately 50% of childhood sellar tumors [10 - 12]. The Childhood Cancer Registry of Piedmont, Italy, has estimated an annual incidence of 1.4 cases per million children [13]. Similar data (e.g., 0.11–0.13 cases per 100,000 people per year) have been reported by other registries in Western countries [14], thus constituting Class III evidence. According to Bunin [14], an annual incidence of 1.3 cases per million people (135 cases of craniopharyngioma) was reported in the USA during the period from 1990 to 1993 (Fig. 1). Among pediatric populations, the incidence is slightly higher. For example, among children aged 5-14 years, the annual incidence is 1.8-2.0 cases per million children (Table 1) [15]. There are no data to support a genetic or environmental basis for this disease. Similar result was also reported by CBTRUS (Central Brain Tumor Registry of the United States) and GDVPTR (Greater Delaware Valley Pediatric Tumor Registry) based on 300 newly diagnosed cases of craniopharyngioma in the USA, irrespective of race. Pediatric cancer registries systematically include intracranial tumors with benign or unspecified behaviors, a category that includes craniopharyngioma [15, 16].

History and Epidemiology

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Table 1. The incidence of craniopharyngioma among various races and sexes in the US population. CBTRUS (1990–1993)

Brain tumor registry of Los Angeles (LAC, 1972–1995)

Cases

Incidence*

Cases

Total

135

0.13

Total

221

Male

68

0.13

Male

118

0.13

Female

67

0.12

Female

103

0.11

Caucasian

109

0.12

Caucasian

62

0.14

African-American

11

0.13

African-American

12

0.12

Caucasian

59

0.11

Male Female

Incidence*

Incidence per million peoples

African-American 11 0.10 *Incidence per million people (The descriptive epidemiology of craniopharyngioma, Neurosurgical Focus, 3(6): Article 1, 1997). CBTRUS, Central Brain Tumor Registry of the United States; LAC, Los Angeles County

2.5 2 1.5 1 0.5 0

0-4

5-9

10-14 15-19 20-34 35-44 45-54 55-64 65-74 75-84

85+

Age

Fig. (1). Incidence of craniopharyngioma during the period from 1990 to 1993 as reported by the Central Brain Tumor Registry of the United States (CBTRUS) [15] (The descriptive epidemiology of craniopharyngioma, Neurosurgical Focus, 3(6): Article 1, 1997).

Although this disease can be detected at any age, even in prenatal and neonatal periods [17, 18], it exhibits a bimodal age distribution with the first peak in children between 5 and 14 years of age and the second in adults older than 65 years [14]. There is Class III evidence of clinical presentation usually in children aged 5–14 years, but there is a second peak in craniopharyngioma incidence in adults older than 75 years [19]. No gender-based differences have been observed in population-based studies from the USA and Finland [14, 20, 21] (Table 3).

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Craniopharyngioma is often associated with an enigmatic and unpredictable growth pattern. Despite the benign histological appearance of this tumor, its frequent tendency to infiltrate critical parasellar structures and aggressive behavior, even after apparently successful therapy, can lead to significant morbidity and mortality, thus posing a considerable medical and social problem. Rutka stated, “There is perhaps no other primary brain tumor that evokes more passion, emotion, and as a result, controversy than does the craniopharyngioma” [21]. Craniopharyngioma is classified as a tumor of the sellar region but may present elsewhere in the central nervous system (CNS) or nasopharynx. Predisposing risk factors (e.g., genetic, environmental) are unknown [11]. Multiple craniopharyngioma cases have been reported within 2 families [22, 23], but there is no data to support a genetic or environmental basis for this disease. Although limited, reports from Asia and Africa indicate a higher incidence of craniopharyngioma relative to Western countries (8). Among 2587 Chinese patients with craniopharyngioma, 586 were aged 0–10 years (22.7%) and 778 were aged 11–20 years (30.1%). Prenatal and neonatal craniopharyngiomas have also been described in multiple reports (2, 9, 11–13, 19, 23). According to our data, which were collected from January 1, 2000 to December 31, 2012, there were 248 cases of craniopharyngioma (age range, 0–72 years) in our department; these accounted for 3.8% of the 6487 total cases of CNS tumors and 18% of the 1365 cases involving sellar region tumors. Regarding pediatric patients, 85 patients presented with craniopharyngioma during childhood (60 M/F ratio= 1.91 1.43 1.33 1.21 2.7 2.0 2.0

Age

Patients Number (cases)

Fig. (2). The prevalence of craniopharyngioma at various ages. The 0–10 year age group accounted for 70 of 248 cases (28.2%), representing the most predominant period of occurrence; 39 (15.7%), 35 (14.1%), 31 (12.5%), 37 (14.9%), 21 (8.5%), and 15 cases (6.1%) occurred in the 11–20, 21–30, 31–40, 41–50, 51–60, and >60-year age groups, respectively. Our data showed significant gender bias; specifically, the prevalence among male patients was significantly higher than that among female patients. The male/female ratios within different age groups ranged from 1.21 to 2.7.

120

Male

n=110 65

n=84 55

60

n=61 37

n=73 41

Female

n=86

n=84

52

48 n=40 23

0

0-10

M/F ratio= 1.44

11-20 21-30 31-40 41-50 51-60 1.53 1.33 1.90 1.54 1.28

>60 Age 1.35

Fig. (3). Data from craniopharyngioma cases at the 10 top hospitals in China; the prevalence of cases at various ages is presented. The predominant period of occurrence was age 0–10 years (110 of 538 cases [20.4%]); in 84 (15.6%), 61 (11.3%), 73 (13.6%), 86 (16.0%), 84 (15.6%), and 40 (7.4%) cases, craniopharyngioma occurred in the 11–20, 21–30, 31–40, 41–50, 51–60, and >60 year age groups, respectively. Our data demonstrated significant gender bias; specifically, the prevalence was significantly higher among men than among women. The male/female ratios in different age groups ranged from 1.28 to 1.9.

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CLINICAL MANIFESTATION The clinical symptoms of craniopharyngioma are due to pressure exerted by the tumor on the surrounding vital structures of the sellar region, including the optical pathway, hypothalamus, pituitary stalk, ventricular system, and pituitary gland. Manifestation severity is closely related to tumor location, size, and potential growth [24 - 27]. Several publications have reported average periods from the presentation of symptoms to diagnosis ranging from 1 week to 372 months [28 31]. The most common presenting manifestations are headache, nausea/vomiting, visual disturbance, growth retardation (in children), and hypogonadism (in adults). Other manifestations are listed in (Table 2A, 2B) [21]. Visual field defects, which usually manifest as bitemporal hemianopsia, are among the most common symptom presentations with an incidence as high as 49% [26, 28, 29]. Notably, this symptom can occasionally be relieved temporarily following cyst rupture and fluid drainage to the ventricles [32]. Other less common features include motor disorders such as hemi- or monoparesis [24, 33 - 35]; seizures [24]; psychiatric symptoms such as emotional lability, hallucinations, paranoid delusions [24, 28, 33, 35]; autonomic disturbances [24]; precocious puberty [29, 35]; inappropriate antidiuretic hormone secretion [36]; chemical meningitis due to spontaneous cyst rupture [37]; hearing loss [38]; anosmia [24]; nasal obstruction [39]; epistaxis [40]; photophobia [41]; emaciation [42, 43]; Weber’s syndrome (ipsilateral III cranial nerve palsy with contralateral hemiplegia consequent to midbrain infarction) [44]; and Wallenberg’s syndrome (consequent to occlusion of the posterior inferior cerebellar artery). Table 2A. Most common presenting clinical manifestations of craniopharyngioma in children and adults (Craniopharyngioma. Endocrine Reviews, 2006, 27(4): 375) [21]. Ref. No. Age (yr)

Headache Nausea/ Papilloedema (%) vomiting(%) (%)

[45]

57

All