Diagnosis and Management of Craniopharyngiomas : Key Current Topics

This Book Provides Up-to-date Coverage Of The Most Relevant Topics In The Diagnosis And Management Of Craniopharyngiomas. After Introductory Discussion Of Natural History And Clinical Presentation, Individual Chapters Are Devoted To Pathological And Molecular Aspects, Use Of Diagnostic Imaging Techniques, The Surgical Approach To Craniopharyngiomas, Radiotherapy And Radiosurgery, And Associated Endocrine Disturbances. A Particular Feature Of The Book Is The Detailed Attention Devoted To The Metabolic Consequences Of The Disease And Related Treatments, Including Obesity And Electrolyte Disturbances, And To Cognitive Alterations. This Book Will Be Of Value To Oncologists, Neurosurgeons, And Endocrinologists By Assisting In Diagnostic Workup, Delivery Of Appropriate Treatment, And Management Of The Serious Metabolic And Endocrine Consequences. Contributors; 1: Craniopharyngiomas: Natural History And Clinical Presentation; 1.1 Introduction; 1.2 History; 1.3 Epidemiology; 1.4 Presentation (clinical And Hormonal Manifestations); References; 2: Craniopharyngioma: Pathological And Molecular Aspects; 2.1 An Historical Note; 2.2 Adamantinous Craniopharyngioma; 2.2.1 Macroscopic Features; 2.2.2 Microscopic Features; 2.2.3 Electron Microscopy; 2.3 Papillary Craniopharyngioma; 2.3.1 Macroscopic Features; 2.3.2 Microscopic Features; 2.3.3 Electron Microscopy; 2.4 The Immunoprofile Of Craniopharyngioma. 2.5 Can Pathology Predict Recurrence?2.5.1 Invasion In Craniopharyngioma And Its Implications; 2.6 Ectopic Locations; 2.7 Metastatic Craniopharyngioma; 2.8 Malignancy In Craniopharyngioma; 2.9 Differential Diagnosis: Lesions Mimicking Craniopharyngioma; 2.10 Craniopharyngioma Can Occur In Association With Other Sellar Lesions; 2.11 Molecular Aspects And Pathogenesis; 2.11.1 Molecular Aetiology Of Adamantinous Craniopharyngioma; 2.11.2 Stem Cells Play A Critical Role In Acp Tumorigenesis; 2.11.3 The Egfr And Shh Pathways Are Upregulated In Mouse And Human Acp. 2.11.4 Molecular Aetiology Of Papillary Craniopharyngiomareferences; 3: Neuroradiological Diagnosis Of Craniopharyngiomas; 3.1 Introduction; 3.2 Imaging Technique; 3.3 General Features; 3.4 Adamantinous Subtype; 3.5 Papillary Subtype; 3.6 Differential Diagnosis; References; 4: Transsphenoidal Approaches To Craniopharyngiomas; 4.1 Introduction; 4.2 Transsphenoidal Approaches; 4.2.1 Microscopic Transsphenoidal Approaches; 4.2.1.1 Sublabial; 4.2.1.2 Endonasal; 4.2.2 Transsphenoidal Transsellar Transdiaphragmatic Approach; 4.2.3 Transsphenoidal Transtuberculum Sellae Approach. 4.2.3.1 Cystic Suprasellar Craniopharyngiomas4.2.3.2 Endoscopic Endonasal; 4.2.4 Anterior Skull Base Endoscopic Approach; 4.2.5 Combined Approaches; 4.3 Endonasal Transsphenoidal Operative Technique; 4.3.1 Preoperative Phase; 4.3.2 Operating Room Layout And Patient Positioning; 4.3.3 Surgical Procedure; 4.3.3.1 Nasal Phase (single Surgeon/two Hands); 4.3.3.2 Sphenoidal Phase (two Surgeons, Three Or Four Hands); 4.3.3.3 Sellar Phase; 4.3.4 Closure; 4.4 Adjuvant Therapy; 4.4.1 Intracavitary Radioisotopes; 4.4.2 Intracavitary Chemotherapy; 4.4.3 Interferon Therapy; 4.5 Outcomes. Conclusionreferences; 5: Surgical Approach To Craniopharyngiomas: Transcranial Routes; 5.1 Introduction; 5.2 Anatomic Consideration For Surgical Planning; 5.3 Surgical Indications; 5.4 Surgical Anatomy; 5.5 Technique; 5.5.1 Foz; 5.5.2 Cista; 5.6 Clinical Series; 5.6.1 Patient Population; 5.6.2 Early Postoperative Results; 5.6.3 Tumor Recurrence; 5.6.4 Further Treatments And Disease Status At Last Follow-up; References; 6: Radiotherapy And Radiosurgery For Craniopharyngiomas; 6.1 Introduction; 6.2 Radiotherapy And Radiosurgery; 6.2.1 Fractionated Stereotactic Radiotherapy. Andrea Lania, Anna Spada, Giovanni Lasio, Editors. Includes Bibliographical References. Contents 6 Contributors 8 1: Craniopharyngiomas: Natural History and Clinical Presentation 10 1.1 Introduction 11 1.2 History 11 1.3 Epidemiology 12 1.4 Presentation (Clinical and Hormonal Manifestations) 12 References 19 2: Craniopharyngioma: Pathological and Molecular Aspects 21 2.1 An Historical Note 21 2.2 Adamantinous Craniopharyngioma 23 2.2.1 Macroscopic Features 24 2.2.2 Microscopic Features 24 2.2.3 Electron Microscopy 26 2.3 Papillary Craniopharyngioma 26 2.3.1 Macroscopic Features 26 2.3.2 Microscopic Features 27 2.3.3 Electron Microscopy 28 2.4 The Immunoprofile of Craniopharyngioma 28 2.5 Can Pathology Predict Recurrence? 30 2.5.1 Invasion in Craniopharyngioma and Its Implications 33 2.6 Ectopic Locations 37 2.7 Metastatic Craniopharyngioma 37 2.8 Malignancy in Craniopharyngioma 38 2.9 Differential Diagnosis: Lesions Mimicking Craniopharyngioma 40 2.10 Craniopharyngioma Can Occur in Association with Other Sellar Lesions 43 2.11 Molecular Aspects and Pathogenesis 45 2.11.1 Molecular Aetiology of Adamantinous Craniopharyngioma 45 2.11.2 Stem Cells Play a Critical Role in ACP Tumorigenesis 47 2.11.3 The EGFR and SHH Pathways Are Upregulated in Mouse and Human ACP 49 2.11.4 Molecular Aetiology of Papillary Craniopharyngioma 52 References 53 3: Neuroradiological Diagnosis of Craniopharyngiomas 63 3.1 Introduction 63 3.2 Imaging Technique 64 3.3 General Features 64 3.4 Adamantinous Subtype 66 3.5 Papillary Subtype 66 3.6 Differential Diagnosis 68 References 73 4: Transsphenoidal Approaches to Craniopharyngiomas 74 4.1 Introduction 74 4.2 Transsphenoidal Approaches 76 4.2.1 Microscopic Transsphenoidal Approaches 78 4.2.1.1 Sublabial 78 4.2.1.2 Endonasal 78 4.2.2 Transsphenoidal Transsellar Transdiaphragmatic Approach 78 4.2.3 Transsphenoidal Transtuberculum Sellae Approach 78 4.2.3.1 Cystic Suprasellar Craniopharyngiomas 79 4.2.3.2 Endoscopic Endonasal 79 4.2.4 Anterior Skull Base Endoscopic Approach 80 4.2.5 Combined Approaches 80 4.3 Endonasal Transsphenoidal Operative Technique 81 4.3.1 Preoperative Phase 81 4.3.2 Operating Room Layout and Patient Positioning 82 4.3.3 Surgical Procedure 83 4.3.3.1 Nasal Phase (Single Surgeon/Two Hands) 83 4.3.3.2 Sphenoidal Phase (Two Surgeons, Three or Four Hands) 84 4.3.3.3 Sellar Phase 84 4.3.4 Closure 85 4.4 Adjuvant Therapy 86 4.4.1 Intracavitary Radioisotopes 86 4.4.2 Intracavitary Chemotherapy 86 4.4.3 Interferon Therapy 86 4.5 Outcomes 87 Conclusion 88 References 88 5: Surgical Approach to Craniopharyngiomas: Transcranial Routes 92 5.1 Introduction 93 5.2 Anatomic Consideration for Surgical Planning 94 5.3 Surgical Indications 95 5.4 Surgical Anatomy 96 5.5 Technique 99 5.5.1 FOZ 99 5.5.2 CISTA 100 5.6 Clinical Series 100 5.6.1 Patient Population 100 5.6.2 Early Postoperative Results 101 5.6.3 Tumor Recurrence 101 5.6.4 Further Treatments and Disease Status at Last Follow-Up 102 References 103 6: Radiotherapy and Radiosurgery for Craniopharyngiomas 107 6.1 Introduction 107 6.2 Radiotherapy and Radiosurgery 109 6.2.1 Fractionated Stereotactic Radiotherapy 110 6.2.2 Radiosurgery 111 Conclusions 116 References 117 7: Endocrine Consequences: Diagnostic Workout and Treatment 119 7.1 GH Deficiency 120 7.1.1 Diagnosis 121 7.1.2 Treatment 122 7.2 Central Hypogonadism 123 7.2.1 Diagnosis 125 7.2.2 Treatment 125 7.2.2.1 Prepubertal Patients 126 Adults Patients 126 7.3 Central Hypoadrenalism 127 7.3.1 Diagnosis 128 7.3.2 Treatment 129 7.4 Central Hypothyroidism 131 7.4.1 Diagnosis 131 7.4.2 Treatment 131 References 132 8: Metabolic Consequences: Obesity and Energy Expenditure, Can They Be Treated? 135 8.1 Introduction 136 8.2 Lifestyle Therapy 136 8.3 Pharmacotherapy 137 8.4 Surgery 144 Conclusions 145 References 145 9: Metabolic Consequences: Electrolyte Disturbances 148 9.1 Introduction 149 9.2 CP and Presurgical Electrolyte Alterations 149 9.3 Postsurgical Electrolyte Alterations 149 9.4 Radiotherapy and Electrolyte Alterations 153 9.5 Diagnosis 153 9.6 Treatment 155 Conclusions 156 References 156 Front Matter....Pages i-viii Craniopharyngiomas: Natural History and Clinical Presentation....Pages 1-11 Craniopharyngioma: Pathological and Molecular Aspects....Pages 13-54 Neuroradiological Diagnosis of Craniopharyngiomas....Pages 55-65 Transsphenoidal Approaches to Craniopharyngiomas....Pages 67-84 Surgical Approach to Craniopharyngiomas: Transcranial Routes....Pages 85-99 Radiotherapy and Radiosurgery for Craniopharyngiomas....Pages 101-112 Endocrine Consequences: Diagnostic Workout and Treatment....Pages 113-128 Metabolic Consequences: Obesity and Energy Expenditure, Can They Be Treated?....Pages 129-141 Metabolic Consequences: Electrolyte Disturbances....Pages 143-153