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J Pediatr Hematol Oncol. 2002 Jun-Jul;24(5):337-42.

Atypical teratoid/rhabdoid tumor of the central nervous system: report on workshop.

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Department of Neurology, Children's National Medical Center, The George Washington University Medical Center, Washington, DC 20010, USA.


Childhood atypical teratoid/rhabdoid tumor (AT/RT) of the central nervous system (CNS) is a recently described entity. Diagnosis is based on distinctive light microscopy and immunohistochemical findings, coupled with molecular genetic analysis. Most AT/RTs demonstrate monosomy 22 or deletions of chromosome band 22q11 with alterations of the hSNF5/INI1 gene. The tumor's incidence is still undefined, but it may comprise as high as 1 in 4 primitive CNS tumors in infants. Treatment is far from optimal, but there are occasional long-term survivors, especially among older children. Therapeutic approached have included surgery, chemotherapy, and radiotherapy. Prospective clinical trials are needed for children with AT/RTs.

[Indexed for MEDLINE]

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