© 1999 by Society
© 1999 by the Society forNeuro-Oncology
Medulloblastoma: Clinical and biologic aspects
Departments of Neurology [R.J.P.], Pediatrics [R.J.P.], Neurosurgery[P.C.], and Diagnostic Imaging [G.V.], Children'sNational Medical Center, Washington, DC 20010; Departments of Neurology [R.J.P.], Pediatrics [R.J.P.], Neurosurgery[P.C.], and Radiology [G.V.], The George WashingtonUniversity Hospital, Washington, DC 20010; and the Department of Pathology, Children's Hospital ofPhiladelphia, University of Pennsylvania, Philadelphia, PA 19104[L.B.R.]
1 Address correspondence and reprint requests to Roger J. Packer, MD, Departmentof Neurology, Children's National Medical Center, 111 Michigan Avenue, NW,Washington, DC 20010.
Medulloblastoma is the most common childhood primary CNS tumor, andtreatment approaches have evolved over the past three decades. The biologicunder-pinnings of medulloblastoma are not fully characterized, but recent workhas identified new, important directions for research. Stratification ofpatients with medulloblastoma into risk groups is the backbone of most ongoingtherapeutic studies. Patients are usually characterized as being eitheraverage risk or poor risk, although an intermediate risk group may exist.Standard treatment for older children with medulloblastoma consists ofradiation and, for most, chemotherapy. Children with nondisseminated diseaseat the time of diagnosis have been reported to have as high as an 80%five-year disease-free survival rate after treatment with reduced dose (2340cGy) craniospinal irradiation, local boost radiation therapy (5500 cGy), andchemotherapy, given during and after radiation therapy. Preradiationchemotherapy has yet to be shown to be of benefit for children withmedulloblastoma. Children with disseminated disease are a highly problematicsubgroup of patients to treat. A variety of new approaches are being studied,most of which are intensifying chemotherapy either prior to or afterradiation. Long-term survivors of medulloblastoma are at significant risk forpermanent endocrinologic, cognitive, and psychological sequelae. Infants andvery young children with medulloblastoma remain a difficult therapeuticchallenge because they have the most virulent form of the disease and are athighest risk for treatment-related sequelae.
Received December 15, 1998; Accepted March 29, 1999
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