Optimal treatment strategy for intracranial germ cell tumors

Object: This study evaluated the long-term outcome of 108 consecutive patients to establish an optimal treatment strategy for respective subgroups of the newly diagnosed intracranial germ cell tumors (GCTs). Methods: A retrospective review of medical records from the authors' department for the duration of April 1989 through March 2007 identified 108 patients with newly diagnosed intracranial GCTs. The diagnosis was germinoma in 83 patients, and nongerminomatous GCT (NGGCT) in 25 patients. Long-term quality of life (QOL) was also evaluated in patients with germinoma. Results: Reading patients with germinoma, the 10-year overall and progression-free survival (PFS) rates at a median follow-up period of 99 months were 86 and 74%, respectively. Patients treated with chemotherapy only demonstrated a decline PFS rate, and patients treated with chemotherapy followed by reduced-dose radiation therapy to the whole ventricle, whole brain, or craniospinal axis indicated significantly improved PFS than patients treated with only radiation or reduced-dose radiation therapy to the focal fields. In the QOL study, the academic outcome for germinoma patients appeared to be better than anticipated. However, neurocognitive defunctionalization was observed at the 10-year follow-up for patients treated with whole brain irradiation. Nongerminomatous GCT patients were categorized into good, intermediate, and poor prognosis groups as proposed by the Japanese Pediatric Brain Tumor Study Group. In the good and intermediate prognosis groups, the 10-year overall survived and PFS rates were 100 and 93%, respectively. In the poor prognosis group, the 3-year overall survived and PFS rates were 56 and 29%, respectively. All patients with NGGCTs, in whom the lesions on MR imaging disappeared after combination therapies consisting of resection, radiation therapy, and chemotherapy, remained alive. Conclusions: Chemotherapy followed by reduced-dose radiation therapy covering the whole ventricle improves the prognosis in patients with germinoma. Combination of radiation therapy, chemotherapy, and radical resection as initial or salvage treatment achieved excellent tumor control in the intermediate prognosis NGGCT group. The outcomes were still dismal for the poor prognosis NGGCT group, so initial therapy should target complete disappearance of all lesions on MR imaging.