Journal of Neuro-Oncology

, Volume 75, Issue 3, pp 309–313

Atypical Teratoid/Rhabdoid Tumors of the Central Nervous System

Childhood Brain Tumors

DOI: 10.1007/s11060-005-6762-8

Cite this article as:
Reddy, A.T. J Neurooncol (2005) 75: 309. doi:10.1007/s11060-005-6762-8

Summary

Atypical teratoid/rhabdoid tumor (AT/RT) is a highly malignant central nervous system neoplasm that usually affects very young children and is typically deadly despite very aggressive treatment. Considered rare, the tumor was not recognized as a distinct entity until the 80’s, due to its similar features with other primitive tumors. Although AT/RT has become increasingly recognized, published data has been based on small series and are retrospective. Based on these data, there are occasional long-term survivors, most of whom received intensive multi-modal therapy. AT/RT is the first pediatric brain tumor for which a candidate tumor suppressor gene has been identified. A mutation or deletion in the INI1 gene occurs in the majority of AT/RT tumors. The function of the gene is not yet understood. Prospective clinical and biologic trials are greatly needed to understand the efficacy of therapeutic interventions, as well as the role of the gene.

Keywords

atypical teratoid/rhabdoid tumors AT/RT malignant childhood brain tumors nervous system embryonal tumors 

Copyright information

© Springer 2005

Authors and Affiliations

  1. 1.The Children’s Hospital of AlbamaUniversity of Alabama at BirminghamBirminghamUSA

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