Abstract
Cerebral atypical teratoid/rhabdoid tumors (AT/RT) of infancy are highly malignant and have a poor prognosis. The authors report on one case with long-term survival. The patient was a 1 year-old boy presenting with a large AT/RT in the right temporal lobe. He was treated with complete surgery, followed by multiagent chemotherapy. Later he had a second resection and intrathecal chemotherapy and Gamma knife radiosurgery was added to the treatment. Except for a well-controlled temporal epilepsy, the boy is doing well after 6 years follow-up. AT/RT should be treated in a multimodal way. Intrathecal chemotherapy and Gamma knife radiosurgery of single recurrent or residual tumors might increase survival.
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