INTRACRANIAL GERM-CELL TUMORS - A COMPREHENSIVE UPDATE OF THE EUROPEAN DATA

Intracranial germ cell tumors are rare tumor entities in childhood and adolescents. Extra- and intracranial germ cell tumors are identical in their histologic pattern and occur in preferential midline localizations such as the pineal and the suprasellar region. Germ cell neoplasms show increasing incidence rates over the last 30 years. The majority of intracranial germ cell neoplasms ate germinomas. About 90 % of the patients with pure germinomas can be salvaged by radiotherapy alone according to modern protocols. Non-germinomatous malignant CNS-germ cell tumors are considered to have a poor prognosis. In order to improve the survival of patients affected by these tumors different treatment approaches adding chemotherapy to conventional surgery and radiotherapy have been initiated by various study groups through-out the world. Due to the rarity of these neoplasms only a very limited number of patients has been enrolled in each study. In 1993 an international working group on these tumors was established by the International Society of Pediatric Oncology (SIOP). The data of the national groups of France, Italy, the United Kingdom and Germany are reviewed to compare the results of the different protocols, to analyse the specific problems of therapy of these neoplasms and to discuss further treatment possibilities in respect to established protocols. For germinomas the data of 87 patients are evaluated. The life-table analysis shows an event-free survival (EFS) of 90 % for patients who received radiotherapy only (46/87), which was craniospinal irradiation in most of the cases, whereas in patients who were treated with a combination of platinum-based chemotherapy and irradiation (30/87) the event-free survival was 92 %. For non-germinomas the combination of platinum-based chemotherapy and craniospinal radiation leads to encouraging results. In respect to the cumulative platinum dosage the Life-table analysis shows an EFS of 86 % for patients with 400 mg/m(2) platinum (17/49). In patients treated with 200mg/m(2) platinum (10/49) the EFS decreases to 56 %.