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Neuro-Oncology 1999 1(2):139-151; doi:10.1093/neuonc/1.2.139
© 1999 by Society
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© 1999 by the Society forNeuro-Oncology

Cytogenetics and molecular genetics of childhood brain tumors

Jaclyn A. Biegel2

Division of Human Genetics and Molecular Biology, The Children'sHospital of Philadelphia and the Department of Pediatrics, University ofPennsylvania School of Medicine, Philadelphia, PA 19104

2 Address correspondence and reprint requests to Jaclyn Biegel, Ph.D., Room 1002Abramson Research Center, The Children's Hospital of Philadelphia, 3516 CivicCenter Boulevard, Philadelphia, PA 19104.


  Abstract

Considerable progress has been made toward improving survival for childrenwith brain tumors, and yet there is still relatively little known regardingthe molecular genetic events that contribute to tumor initiation orprogression. Nonrandom patterns of chromosomal deletions in several types ofchildhood brain tumors suggest that the loss or inactivation of tumorsuppressor genes are critical events in tumorigenesis. Deletions ofchromosomal regions 10q, 11 and 17p, for example, are frequent events inmedulloblastoma, whereas loss of a region within 22q11.2, which contains theINI1 gene, is involved in the development of atypical teratoid and rhabdoidtumors. A review of the cytogenetic and molecular genetic changes identi-fiedto date in childhood brain tumors will be presented.

Received December 18, 1998; Accepted January 7, 1999


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