[Primary germ cell tumors of the central nervous system]

Germ cell tumors located in the central nervous system represent less than 4% of the intracranial tumors and affect primarily children and young adults. They are located usually in the pineal and the suprasellar region. The most frequent histological type is germinoma (65%) and it holds the best prognosis. The non-seminomatous tumors, on the other hand, are an heterogeneous group, more aggressive and less responsive to treatment. The diagnosis requires a combination of radiology, tumor markers, CSF cytology and histological confirmation with biopsy. The role of surgery has to be restricted to the obtention of material for diagnosis. The radiotherapy (RT) has traditionally been considered as the standard treatment and it provides 5 year survival rates of 30-88%. Chemotherapy (CT), using cisplatin-based regimens, has been gradually introduced over the last few years. Germinoma is highly sensible to CT and achieves a high rate of complete responses (66-86%), as the initial treatment prior to RT as well as in relapses postRT. RT alone provides suboptimal results in the treatment of non-seminomatous cranial germ cell tumors, therefore, combined treatment of CT and RT with higher radiation dosage seems to be an advantageous alternative to RT alone. As a conclusion, the results obtained in recent trials open up important expectations in the management of these tumors. The inclusion of CT has allowed a reduction in the doses of RT in germinoma and an improvement in the results in non-seminoma tumors. Further and better studies are necessary to define the best strategies, as well as the possibility to introduce schedules with only CT.