SECRETING GERM-CELL TUMORS OF THE CENTRAL-NERVOUS-SYSTEM (CNS) - FIRST RESULTS OF THE COOPERATIVE GERMAN ITALIAN PILOT-STUDY (CNS SGCT)/

Background Secreting germ cell tumors are an invariably fatal subgroup within the malignant pediatric brain tumors. Thus in 1993, an interna tional working group was initiated to establish a cooperative study fo r diagnostic and treatment of intracranial secreting germ cell tumors of the CNS. To pilot this protocol, since 1994 German and Italian pati ents are treated in accordance with the established guidelines. Method s Regarding to the achieved consensus within the international protoco l commitee a characteristical diagnostic imaging (CT/MRI scan) of head and spine and a significant increase of tumor markers beta-HCG (> 50 IU/l) and/or AFP (>25 ng/ml) are defined as sufficient diagnostic crit eria. Additionally staging procedures include an initial CSF cytology. Treatment consists of 4 courses PEI: platinum (20 mg/m(2) day 1-5), V P16 (100 mg/m(2) day 1-3), and ifosfamide (1.5 g/m(2) day 1-5), Surger y m of the residual tumor is administered after chemotherapy, if resec tion is possible. followed by craniospinal irradiation (30 Gy with tum or boost 24 Gy). Results Until September 96, 19 patients (16 boys and 3 girls) aged 8 to 19 years are registered and have finished their tre atment. Seven children are diagnosed by elevated tumor markers, Six of 7 children with stereotactic fine needle biopsy and histology of germ inoma have a significant marker increase as a specific characteristic for secreting non-germinomatous germ fell tumors. In 6 patients, the t umor is primarily resected, 2 children are biopsied, In 2 children spi nal metastases are diagnosed initially. Tumor marker response is evalu ated in 16 children. Thirteen of 16 patients show a clear marker norma lization after 2 courses of PEI. One boy with a slight increase elf th e tumor marker after the 4th course developed an early spinal relapse and died, One girl showed a spinal recurrence during focal radiotherap y. She is still under relapse treatment. A significant decrease of tum or volume after chemotherapy is documented In 10/13 children, who have a definite signal tumor at start of therapy. In 3 children tumor volu me does not change despite of marker normalization. Histology of these tumors is teratoma. One of these children died postoperatively becaus e of tumor bleeding. 17/19 patients are alive, 16 of them are in compl ete remission with a median follow-up of 11 months. Conclusion These r esults show a further significant increase of event-free survival (EFS 81%). The piloted chemotherapy is proven to be effective and the prot ocol is now open as an international SIOP CNS GCT study that is starte d in October 1996.