Central nervous system germ cell tumors: an update.

Abstract

Malignant germ cell tumors in the central nervous system are rare and not well known because they occur mainly in adolescents, an age in which patients are dispersed in adult and pediatric wards. Their biology starts to be unraveled by high-throughput genomics and their treatment is now well defined thanks to international studies, including patients from childhood through adulthood. Chemotherapy gained definitively a role apart from radiotherapy in order to improve tumor control in secreting neoplasms, but also to decrease the volume or dose of radiation therapy in germinomas. Neurocognitive outcome remains good with some disparities because of tumor location, patients with pineal tumors being less impaired than those with supratentorial malignant germ cell tumors. Alterations in the KIT/RAS as well as in the mTOR/AKT pathways have been frequently reported and could represent interesting opportunities to introduce targeted therapies in these neoplasms. Diagnosis and treatment of malignant germ cell tumor of the brain are now well established within experienced multidisciplinary teams taking care of adolescents and young adults. The prognosis of secreting tumors has not yet reached the excellent results obtained for germinomas but aggressive chemotherapy including high-dose chemotherapy with stem-cell support may contribute to improve their outcome. The role of targeted therapies has yet to be determined in view of the recently described molecular findings.