Rhabdoid tumors

Rhabdoid tumors are rare and extremely aggressive tumors of early childhood. They occur in a number of locations: 

This article touches on the generalities of these tumors, but each is then discussed separately. 


Almost all reported cases of rhabdoid tumors, regardless of site of origin, occur in young children, between birth and 2 years of age 4

In approximately 10% of cases of renal malignant rhabdoid tumors, patients then subsequently develop intracranial atypical teratoid/rhabdoid tumor (AT/RT) 2


Irrespective of site of origin, rhabdoid tumors share deletions in the long arm of chromosome 22, mapped to the INI1 gene, believed to be a tumor suppressor 1,4

These tumors contain characteristic rhabdoid cells, so-called on account of the light microscopy resemblance to skeletal muscle cells 2

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Article information

rID: 12421
System: Paediatrics
Synonyms or Alternate Spellings:
  • Rhabdoid tumors

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Cases and figures

  • Atypical teratoid rhabdoid tumor (AT/RT)
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