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Neurooncol Pract. 2017 Sep;4(3):138-150. doi: 10.1093/nop/npx011. Epub 2017 Aug 11.

Case-based review: pediatric medulloblastoma.

Author information

1
Division of Hematology/Oncology, Department of Pediatrics, University of California, San Francisco, 550 16th Street, 4th Floor, San Francisco, CA 94158 (C.K., S.M.); Center for Neuroscience and Behavioral Medicine, Children's National Medical Center, 111 Michigan Avenue NW, Washington, DC 20010 (R.P.); Brain Tumor Institute, Children's National Medical Center, 111 Michigan Avenue NW, Washington, DC 20010 (R.P., E.H.); Division of Hematology/Oncology, Center for Cancer and Blood Disorders, Children's National Health Systems, 111 Michigan Avenue NW, Washington, DC 20010 (R.P., E.H.); Department of Radiation Oncology, University of California, 1825 4th Street, San Francisco, San Francisco, CA 94158 (D.R., S.B.); Department of Neurological Surgery, University of California, San Francisco, 505 Parnassus Avenue, M779, San Francisco, CA 94143 (C.R., S.M.); Dana-Farber/Boston Children's Cancer and Blood Disorders Center, 450 Brookline Avenue, Boston, MA 02215 (P.B.); Department of Pediatrics, Harvard Medical School, Boston, MA 02215 (P.B.); Broad Institute of MIT and Harvard, 415 Main Street, Cambridge, MA 02142 (P.B.); Division of Neuropathology, Department of Pathology, University of California, San Francisco, 505 Parnassus Avenue, M551, Box 0102 San Francisco, CA 94143 (D.S.); Department of Radiology, University of California, San Francisco, 550 Parnassus Avenue, M327, San Francisco, CA 94143 (M.A., S.C.); Department of Neurology, Neurosurgery and Pediatrics, University of California, San Francisco, 550 Sandler Neurosciences, 625 Nelson Rising Lane, 402B, Box 0434, San Francisco, CA 94158 (S.M.).

Abstract

Medulloblastoma is the most common malignant brain tumor affecting children. These tumors are high grade with propensity to metastasize within the central nervous system and, less frequently, outside the neuraxis. Recent advancements in molecular subgrouping of medulloblastoma refine diagnosis and improve counseling in regards to overall prognosis. Both are predicated on the molecular drivers of each subgroup-WNT-activated, SHH-activated, group 3, and group 4. The traditional therapeutic mainstay for medulloblastoma includes a multimodal approach with surgery, radiation, and multiagent chemotherapy. As we discover more about the molecular basis of medulloblastoma, efforts to adjust treatment approaches based on molecular risk stratification are under active investigation. Certainly, the known neurological, developmental, endocrine, and psychosocial injury related to medulloblastoma and its associated therapies motivate ongoing research towards improving treatment for this life-threatening tumor while at the same time minimizing long-term side effects.

KEYWORDS:

chemotherapy; medulloblastoma; radiation; risk stratification; targeted therapy

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