Journal of Neuro-Oncology

, Volume 24, Issue 1, pp 21–28

Central nervous system atypical teratoid/rhabdoid tumors of infancy and childhood

  • Lucy Balian Rorke
  • Roger Packer
  • Jaclyn Biegel
Article

Summary

Clinical and morphological features of an apparently unique, biologically aggressive central nervous system tumor in 32 infants and children are presented. This neoplasm is formed wholly or partly by rhabdoid cells, areas resembling typical primitive neuroectodermal tumor, and, less frequently, malignant mesenchymal and/ or epithelial tissue. The tumor has been named atypical teratoid/rhabdoid tumor (ATT/RhT) and is regarded as a unique class of primary central nervous system (CNS) tumors. It occurs most commonly in infants less than two years of age, has often metastasized throughout the CNS at presentation, does not respond to therapy and causes death less than a year after diagnosis. These tumors may occur in any CNS site but almost 60% are located in the cerebellum. The most common chromosomal abnormality involves chromosome 22.

Key words

atypical teratoid tumor rhabdoid tumor primitive neuroectodermal tumor medulloblastoma 

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Copyright information

© Kluwer Academic Publishers 1995

Authors and Affiliations

  • Lucy Balian Rorke
    • 1
  • Roger Packer
    • 2
  • Jaclyn Biegel
    • 1
  1. 1.The Children's Hospital of PhiladelphiaPhiladelphiaUSA
  2. 2.Washington, D.C. Children's HospitalWashington DCUSA
  3. 3.The Children's Hospital of PhiladelphiaPhiladelphiaUSA

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