Primary germinal tumors of the nervous system in children and adolescents. A retrospective study of 35 cases from 1975 to 1987

Thirty-five cases of primary intracranial germ cell tumor treated at the Institut Gustave-Roussy between 1975 and 1987 were reviewed. There were 23 males and 12 females; the median age was 12 years (range: 3 months to 17 years). Tumors originated in the pineal region in 15 cases, in the suprasellar region in 15 cases, and in both region in 5 cases. The diagnosis was confirmed histologically in 20 cases (14 germinomas and 6 non germinomatous germ cell tumors), and cytologically in 5 cases. There were 11 secreting tumors (alpha-foetoprotein or chorionic gonadotrophin) without histological diagnosis in 6 cases. Four patients were treated with a presumptive diagnosis. Surgical resection was performed in 6 cases. Thirty-two patients were irradiated on the tumor site; 17 received a craniospinal irradiation and 7 a cranial irradiation. Six patients received chemotherapy. Twenty-five patients are alive with a median follow-up of 4 years. Prognosis is better in germinomas than in non germinomatous germ cell tumors (survival rates = 88 and 50% respectively). Chemotherapy is presently being evaluated in the treatment of germ cell tumors with the aim of using lower doses of radiotherapy and decreasing its late effects in germinomas and of improving the prognosis of other histological types.