Abstract
Central nervous system germ cell tumors (CNSGCTs) are tumors of children and young adults. They affect mainly the pineal body, neurohypophysis (“suprasellar” area) and basal ganglia, but can arise in any other CNS location. Histologically, they are divided into germinoma and non-germinomatous germ cell tumors (NGGCTs), the latter including teratoma, embryonal carcinoma, choriocarcinoma, and yolk sac tumor. Teratomas are further subclassified into mature teratoma, immature teratoma, and teratoma with malignant transformation. Except for mature teratoma, all of these tumors are biologically malignant. Histologically, they resemble reproductive cells, fetal tissues, or related tissues such as placenta, and mixtures of these tumor subcategories are not rare. Initial symptoms include increased intracranial pressure due to obstructive hydrocephalus, abnormality of ocular movement and endocrine disorders. Some tumors of these subclasses produce humoral tumor markers, beta-human chorionic gonadotropin, alpha-fetoprotein or other markers, which can be used for differential diagnosis or clinical follow-up. Only mature teratoma can be cured with surgery alone, and the other tumors require adjuvant therapy for complete remission. Germinomas respond well to irradiation and can be cured, but platinum-based chemotherapy combined with reduced-dose irradiation has been recently used to minimize radiation-related toxicities or complications. As the other tumors cannot be cured by irradiation alone, more intensive platinum-based chemotherapy and radiation therapy including whole-neuroaxis irradiation are employed. Although better disease control and longer survival can now be achieved, the results of treatment are still not satisfactory. Therefore, development of new treatments, and improvements to existing ones, are needed for CNSGCTs.
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Fujimaki, T. (2012). Central Nervous System Germ Cell Tumor. In: Hayat, M. (eds) Tumors of the Central Nervous System, Volume 4. Tumors of the Central Nervous System, vol 4. Springer, Dordrecht. https://doi.org/10.1007/978-94-007-1706-0_7
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