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The Oncologist, Vol. 8, No. 2, 174–186, April 2003
© 2003 AlphaMed Press

ORIGINAL PAPER
Pediatric Oncology

Advances in the Diagnosis, Molecular Genetics, and Treatment of Pediatric Embryonal CNS Tumors

Tobey J. MacDonalda, Brian R. Rooda, Maria R. Santib, Gilbert Vezinac, Kimberly Bingamand, Philip H. Cogend, Roger J. Packere

a Departments of Hematology/Oncology, b Pathology, c Radiology, d Neurosurgery, and e Neurology, Children’s Hospital National Medical Center, Washington, DC, USA

Correspondence: Tobey J. MacDonald, M.D., Children’s Hospital National Medical Center, Department of Hematology/Oncology, 111 Michigan Avenue, NW, Washington, DC 20010, USA. Telephone: 202-884-2800; Fax: 202-884-5685; e-mail: tmacdona{at}cnmc.org

Embryonal central nervous system (CNS) tumors are the most common group of malignant brain tumors in children. The diagnosis and classification of tumors belonging to this family have been controversial; however, utilization of molecular genetics is helping to refine traditional histopathologic and clinical classification schemes. Currently, this group of tumors includes medulloblastomas, supratentorial primitive neuroectodermal tumors, atypical teratoid/rhabdoid tumors, ependymoblastomas, and medulloepitheliomas. While the survival of older children with nonmetastatic medulloblastomas has improved considerably within the past two decades, the outcomes for infants and for those with metastatic medulloblastomas or other high-risk embryonal CNS tumors remain poor. It is anticipated that the emerging field of molecular biology will greatly aid in the future stratification and therapy for pediatric patients with malignant embryonal tumors. In this review, recent advances in the diagnosis, molecular genetics, and treatment of the most common pediatric embryonal CNS tumors are discussed.

Key Words. Primitive neuroectodermal tumor • Medulloblastoma • Atypical teratoid/rhabdoid tumor • Diagnosis • Molecular genetics • Treatment




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