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Atypical teratoid-rhabdoid tumor

An atypical teratoid/rhabdoid tumor, or ATRT, is generally found in the cerebellum of children younger than two. It is very rare, very aggressive, and spreads throughout the central nervous system. About one to two percent of children with brain tumors have the atypical teratoid/rhabdoid tumor type.

Treatment involves the surgical excision of the tumor followed with chemotherapy. In children who are a little older, radiation therapy may be an option in addition to surgery and chemo. Young children treated with radiation may have permanent problems with thinking, learning, and growing. Chemotherapy is often used as a delaying tactic for very young children, staving off the growth of the atypical teratoid/rhabdoid tumor until the child is old enough for the brain to be a little more resilient to radiation therapy.

Survival of children younger than three at diagnosis is less than ten percent. Older children treated with surgery followed by chemotherapy and radiation therapy do better long-term, with about 70% survival. Some new radiation therapies look promising as well, and stem cell transplantation may offer new avenues of treatment.

Web Resources On Atypical teratoid-rhabdoid tumor

Atypical teratoid/rhabdoid tumors
Central Nervous System Atypical Teratoid/Rhabdoid Tumor

Book Resources On Atypical teratoid-rhabdoid tumor

Brain Tumors in Children: Principles of Diagnosis and Treatment by Cohen & Duffner
Pediatric CNS Tumors by Nalin Gupta et al

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