Atypical Teratoid Rhabdoid Tumor

  • Adekunle M. Adesina
  • J. Hunter
  • L. Rorke-Adams
Chapter

Abstract

Atypical teratoid/rhabdoid tumors are malignant high grade embryonal (WHO Grade IV) tumors seen in children often below the age of 3 years and rarely above the age of 6 years. Account for 10% of tumors in infants with slight male predominance, M:F ratio = 2:1 Characteristically poorly differentiated, contain rhabdoid cells with divergent differentiation to form epithelial, mesenchymal, neuronal, and glial components and may be associated with a subpopulation of primitive neuroectodermal cells.

Keywords

Atypical teratoid rhabdoid tumor PNET Embryonal tumor Rhabdoid predisposition syndrome 

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Copyright information

© Springer Science+Business Media, LLC 2010

Authors and Affiliations

  • Adekunle M. Adesina
    • 1
  • J. Hunter
    • 2
  • L. Rorke-Adams
    • 3
  1. 1.Section of Neuropathology and Molecular Neuropathology Laboratory, Department of PathologyTexas Children’s Hospital, Baylor College of MedicineHoustonUSA
  2. 2.Department of RadiologyTexas Children’s Hospital, Baylor College of MedicineHoustonUSA
  3. 3.Department of PathologyThe Children’s Hospital of PhiladelphiaPhiladelphiaUSA

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