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

Roger J. Packer, Jaclyn A. Biegel, Susan Blaney, Jonathan Finlay, J. Russell Geyer, Richard Heideman, Joanne Hilden, Anna J. Janss, Larry Kun, Gilbert Vezina, Lucy B. Rorke, Malcolm Smith

Research output: Contribution to journalArticlepeer-review

202 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)337-342
Number of pages6
JournalJournal of Pediatric Hematology/Oncology
Issue number5
StatePublished - 2002


  • Atypical teratoid tumors
  • Chemotherapy
  • Infantile brain tumors
  • Radiotherapy
  • Rhabdoid tumors

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Hematology
  • Oncology


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