Brain metastases in children and adolescents with extracranial germ cell tumor - data of the MAHO/MAKEI-registry.

Background: We analyzed 15 children and adolescents with extracranial germ cell tumor (GCT) and brain metastases reported to the MAHO/MAKEI registry. Patients and Methods: Between 1982 and 2009, 2 077 patients were prospectively enrolled onto the MAHO/MAKEI studies (overall survival: 0.88 +/- 0.03). All patients with advanced malignant GCTs received cisplatin-based chemotherapy (overall survival: 0.81 +/- 0.04 (734/823). Results: 15 patients with brain metastases were reported; in 6 of them at diagnosis and 9 respectively during follow-up (6weeks-28months after end of therapy, mean = 10 months). Most patients were male (13/15) and adolescent (10/15). 8 patients suffered from mediastinal GCTs. Pure Choriocarcinoma (CC) or CC in combination with other histologies was diagnosed in 12 patients. Clinical symptoms were reported in most patients. In all patients with secondary brain metastases the previously normalised tumor markers AFP and/or HCG increased again prior to the onset of neurological symptoms. Only 1 of the patients with primary brain metastases survived, whereas 4 of 9 with secondary metastases are in remission after additional treatment. Conclusion: The risk for intracranial metastases increases with age, male gender and mediastinal or testicular primary site and choriocarcinoma histology. Development of neurological symptoms at initial diagnosis or during follow-up should lead to rapid clinical re-evaluation including CNS imaging and assessment of tumor markers. Treatment of brain metastases includes intensified chemotherapy and surgical resection, irradiation has to be considered in special clinical situations.