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Embryonal Brain Tumors

AbstractEmbryonal brain tumors are a heterogeneous group of neoplasms that primarily occur in infants and young children. They are highly cellular tumors with brisk mitotic activity, and they share a propensity for dissemination throughout the neuroaxis. Emerging molecular data enable improved diagnostic and prognostic discrimination for these tumors. Because of their aggressive potential, they are treated similarly with multimodality therapy including maximal safe resection, chemotherapy, and age- and risk-adapted radiotherapy. Craniospinal irradiation is commonly used in the treatment of these patients, especially in those older than 3 years. Because proton therapy allows for increased sparing of the anterior structures in craniospinal irradiation, there is a particular interest in using proton therapy to treat these young patients. For very young patients treated with focal fields only, proton therapy also decreases unnecessary radiation exposure to uninvolved intracranial structures. It is hoped that the use of proton therapy for these vulnerable patients will translate into decreased long-term neurocognitive, endocrine, vascular, and developmental effects, in addition to a decreased risk of second malignancies. This review describes the role of radiation in general and proton therapy in particular for the treatment of medulloblastoma, central nervous system primitive neuroectodermal tumors, atypical teratoid/rhabdoid tumors, and the recently described embryonal tumor with multilayered rosettes.