CNS germ cell tumors: a clinical review and future directions

Abstract

CNS germ cell tumors (GCT) are rare tumors that arise in midline brain regions (mostly pineal or suprasellar). They are of two types, germinoma and nongerminomatous GCT (NGGCT) which include teratoma, choriocarcinoma, yolk sac, embyronal carcinoma and mixed GCT. Tissue is needed for diagnosis unless serum or cerebrospinal fluid markers, b-HCG or AFP, are elevated. Germinomas can be cured with radiation therapy (RT) alone (whole ventricle fields, if localized), but chemotherapy may permit RT dose-reduction. Best outcomes for NGGCT are with RT and chemotherapy. Craniospinal RT is needed for all disseminated tumors and best survival for localized NGGCT has included craniospinal RT. Recent genetic findings in CNS GCT may lead to therapies targeting their oncogenic pathways.