Main description:

Intracranial germ cell tumors are a group of uncommon neoplasms of the central nervous system. The clinical features and natural history of these lesions are quite unique and variable. While intracranial germ cell tumors have been a fascination to neurooncologists for decades, the relatively small number of patients seen in any single institution has hampered the important clinical investigation that is so needed.
This text is complete with detailed information concerning the epidemiology, pathology, oncological biology, clinical findings, radiology, and treatment options including surgical strategy, radiotherapy, and chemotherapy for this heterogeneous group of neoplasms. The ongoing clinical trials concerning the optimization of therapy are efficiently summarized. An important final segment addresses the late sequelae of therapy which is of great significance since the majority suffering from these tumors are young patients.
This first and only book on intracranial germ cell tumors includes excellent and comprehensive data sheets, illustrations, and radiograms. It provides a detailed and outstanding reference source for physicians taking care of patients with intracranial germ cell tumors, and will be a very welcome edition to their reference libraries.

Contents:

I. Introduction.- II. WHO histological classification of tumors of the central nervous system (CNS): germ cell tumors (GCTs).- III. Epidemiology of CNS GCTs.- 1. Histological variation and incidence.- 2. Racial and geographical factors.- 3. Age factor.- 4. Gender factor.- 5. Anatomical factor.- 6. Genetic factor.- 7. Other factors.- IV. Pathology.- 1. General features of GCTs.- 2. Germinoma.- 3. Embryonal carcinoma.- 4. Yolk sac tumor (Endodermal sinus tumor).- 5. Choriocarcinoma.- 6. Teratomas.- 6.1 Mature teratoma.- 6.2 Immature teratoma.- 6.3 Teratoma with malignant elements.- V. Immunobiology of tumor infiltrating lymphocytes (TIL) in germinomas.- 1. Cell types of germinoma TIL.- 2. The cause and role of immune cell infiltration in germinomas.- 3. Cytotoxicity of TIL to tumor cells.- 4. Conclusive remarks.- VI. Genesis and genetics of intracranial germ cell tumors.- 1. Clonal evolution model for tumorigenesis.- 2. Oncogenes and tumor suppressor genes.- 3. Problematic of ontogenesis of CNS and extracranial GCTs.- 3.1 All cells originate from primordial germ cells.- 3.2 All cells originate from different precursors during early embryonic development.- 3.3 Primordial germ cells have a physiological role outside the gonads.- 3.4 All cells originate from early toti- or pluri-potent precursor cells.- 4. Oncogenesis of GCTs.- 4.1 Oncogenesis of extracranial GCTs.- 4.2 Oncogenesis of CNS GCTs.- 4.2.1 Cytogenetic findings.- 4.2.2 Genomic studies.- VII. Clinical findings and diagnosis.- 1. Neurological manifestations.- 1.1 Neurological signs and symptoms.- 1.2 Disturbances of vertical eye movements.- 1.2.1 Posterior commissure.- 1.2.2 Rostral interstitial nucleus of the medial longitudinal fasciculus (riMLF).- 1.2.3 Interstitial nucleus of Cajal and periaqueductal gray matter.- 1.3 Signs and symptoms of the tumors in unusual sites.- Radiological diagnosis.- 2.1 Overview of the imaging of brain tumors.- 2.2 Imaging of CNS GCTs.- 2.2.1 Germinomas.- 2.2.2 Teratomas.- 2.2.3 Choriocarcinoma.- 2.2.4 Yolk sac tumor (Endodermal sinus tumor).- 2.2.5 Embryonal carcinoma.- 2.2.6 Mixed GCTs.- 2.3 Differential diagnosis.- 2.3.1 Pineal region.- 2.3.2 Sellar and suprasellar region.- 2.3.3 Basal ganglia and thalamus.- 3. Endocrinological manifestations.- 3.1 Diabetes insipidus.- 3.2 Anterior pituitary hormone deficits.- 3.3 Endocrine evaluation.- 3.4 Precocious puberty.- 4. Ophthalmological manifestations.- 4.1 Visual symptoms caused by suprasellar GCTs.- 4.2 Visual field defects caused by germinoma.- 4.3 Prognosis of visual disturbance.- 5. Tumor markers.- 5.1 Alpha-fetoprotein.- 5.2 Human chorionic gonadotropin.- 5.3 Human placental alkaline phosphatase.- 5.4 Carcinoembryonic antigen.- 5.5 Use of the tumor marker as a predictor of treatment efficacy.- 5.6 Detection of tumor relapse by tumor markers.- VIII. Prognosis of CNS GCTs.- 1. Survival and recurrence after therapy.- 1.1 Germinomas.- 1.2 Teratomas.- 1.3 Other malignant subtypes.- 2. Outcome of patients.- 2.1 Performance status in survivors.- 2.2 Requirement of hormone replacement therapy.- 2.3 Radiation-induced brain injury, vessel occlusion, and neoplasm.- IX. Overview of management.- 1. Classification of CNS GCTs for management.- 2. Diagnosis to select an appropriate treatment mode.- 3. Overview of treatment.- 3.1 Good prognosis group.- 3.2 Technique of small field irradiation after preradiation chemotherapy.- 3.3 Intermediate prognosis group.- 3.4 Poor prognosis group.- 4. Preliminary results of Japanese Cooperative Study.- X. Surgical management.- 1. Surgical strategy for germinomas.- 2. Surgical approaches to tumours of the pineal region.- Surgical anatomy.- Dural sinuses and veins.- Brain structures.- Arteries.- Cisterns.- Choice of approach.- Surgical techniques.- Occipital transtentorial approach.- Infratentorial supracerebellar approach.- Complications.- 3. Stereotactic biopsy.- 3.1 Indications.- 3.2 Technical choices.- 3.3 Results.- 3.4 Stereotactic biopsy and the role of radiosurgery.- XI. Chemotherapy for CNS GCTs.- 1. Overall view of chemotherapy for CNS GCTs.- 1.1 Rationale.- 1.2 Use of empirical chemotherapy for presumed CNS GCTs.- 1.3 Chemotherapy for CNS metastases from systemic GCTs.- 1.4 Chemotherapy for extraneural metastases of central nervous system GCTs.- 2. Chemotherapy for recurrent CNS GCTs.- 2.1 Treatment for GCTs recurrent after irradiation.- 2.2 Treatment for GCTs recurrent after chemotherapy.- 3. Chemotherapy for newly-diagnosed CNS GCTs: neoadjuvant chemotherapy.- 3.1 General.- 3.2 Neoadjuvant chemotherapy for CNS germinomas.- 3.3 Neoadjuvant chemotherapy for non-germinomatous GCTs.- 3.4 Prospective multicenter trials of neoadjuvant chemotherapy for CNS GCTs.- 3.4.1 Germinomas.- 3.4.2 Non-germinomatous GCTs.- 3.5 Sandwich therapy for non-germinomatous GCTs: delayed surgical resection.- 3.6 The significance of residual masses after chemotherapy treatment.- 4. Chemotherapy - only trials.- 4.1 Germinomas.- 4.2 Non-germinomatous GCTs.- 5. Predictors of outcome to treatment.- 6. Myeloablative chemotherapy followed by autologous stem cell rescue for CNS GCTs.- 7. A view of the future.- XII. Radiotherapy for CNS GCTs.- 1. "Diagnostic radiotherapy" or radiation test.- 2. Radiotherapy for CNS germinoma.- 2.1 History of the radiotherapy for CNS germinoma.- 2.2 Dose required to control primary tumor.- 2.2.1 Critical review of clinical studies.- 2.2.2 Dose reduction trials.- 3. Irradiation volume and technique.- 3.1 Craniospinal irradiation.- 3.1.1 Indication for CNS germinoma.- 3.1.2 Technical consideration for craniospinal irradiation.- 3.2 Smaller field irradiation.- 3.3 Low dose small field irradiation following to chemotherapy.- 3.4 HCG-producing CNS germinoma.- 4. Radiotherapy for CNS non-germinomatous GCTs.- 4.1 Mature teratoma.- 4.2 Immature teratoma.- 4.3 Teratoma with malignant transformation.- 4.4 Highly malignant non-germinomatous GCTs.- 4.5 Mixed GCTs.- 5. Stereotactic irradiation (STI).- 6. Future remarks.- XIII. Adverse effect of therapy and late sequelae in survivors.- 1. Effect of external radiotherapy.- 1.1 Physical development.- 1.2 Neuropsychological sequelae.- 1.2.1 Histopathological findings.- 1.2.2 General observation after radiotherapy for children.- 1.2.3 Findings after radiotherapy for CNS GCTs.- 1.2.4 Prevention and follow-up.- 2. Induction of secondary tumors after irradiation.- 2.1 The molecular basis.- 2.2 General features.- 2.3 Relative risks.- 2.4 Radiation-induced meningiomas, astrocytic tumors, and sarcomas.- 3. Hypothalamic-pituitary dysfunction and their management.- 3.1 Growth hormone deficiency.- 3.2 Pubertal development.- 3.3 Other hypothalamic-pituitary disorders.- 3.4 Mechanism and site of the lesion.- 3.5 Growth.- 3.6 Treatment.- 4. Ototoxicity of chemotherapy.- 4.1 Cisplatin-induced ototoxicity.- 4.2 Audiological monitoring.- 4.3 Prevention and follow-up.- 5. Clinical significance of pineal gland dysfunction.- 5.1 Revisit to the pineal gland.- 5.2 Abnormal melatonin secretion.- 5.3 Disorders of sleep and sexual development.- 5.4 Aging and cancer.