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Introduction
Medulloblastoma, or PNET of the posterior fossa, is the most common malignant brain tumor of childhood. Medulloblastomas typically present in the first decade of life with signs and symptoms of obstructive hydrocephalus. A few decades ago, medulloblastoma was considered a likely fatal disease. Today, with the combined modalities of surgery, radiation therapy, and chemotherapy, long-term survival is the norm rather than the exception. Future therapies will focus on maintaining or improving long-term survival rates while minimizing the toxicities and late effects associated with current treatment regimens.
Key Points
- Management of associated hydrocephalus: Timely surgical resection alone usually obviates need for CSF diversion. Preoperative ETV is an option but is a point of current controversy. Preoperative shunt placement should be avoided.
- Overall management multimodal: Maximum surgical resection should be performed, but it is preferable to leave behind tumor that is adherent to the brainstem. Postoperatively, craniospinal radiation therapy (except in infants) and chemotherapy are indicated.
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