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 i n their histologic pattern and occur in preferential midline localizat ions such as the pineal and the suprasellar region. Germ cell neoplasm s 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 a ccording to modern protocols. Non-germinomatous malignant CNS-germ cel l 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 radiotherap y have been initiated by various study groups through-out the world. D ue to the rarity of these neoplasms only a very limited number of pati ents 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 res ults of the different protocols, to analyse the specific problems of t herapy of these neoplasms and to discuss further treatment possibiliti es in respect to established protocols. For germinomas the data of 87 patients are evaluated. The life-table analysis shows an event-free su rvival (EFS) of 90 % for patients who received radiotherapy only (46/8 7), which was craniospinal irradiation in most of the cases, whereas i n patients who were treated with a combination of platinum-based chemo therapy and irradiation (30/87) the event-free survival was 92 %. For non-germinomas the combination of platinum-based chemotherapy and cran iospinal radiation leads to encouraging results. In respect to the cum ulative platinum dosage the Life-table analysis shows an EFS of 86 % f or patients with 400 mg/m(2) platinum (17/49). In patients treated wit h 200mg/m(2) platinum (10/49) the EFS decreases to 56 %.