Encyclopedia of Cancer

Living Edition
| Editors: Manfred Schwab

Rhabdoid Tumor

Living reference work entry
DOI: https://doi.org/10.1007/978-3-642-27841-9_5093-2

Definition

A highly malignant tumor that primarily affects infants and young children. Rhabdoid cells contain large nuclei with prominent nucleoli and abundant eosinophilic cytoplasm that often contains filamentous cytoplasmic inclusions. In tumors of the central nervous system (CNS), areas of rhabdoid cells may be seen in juxtaposition to areas of primitive neuroepithelial cells resembling primitive neuroectodermal tumor, as well as mesenchymal tissue and/or epithelial tissue. The designation atypical teratoid/rhabdoid tumor (AT/RT) is used to describe the CNS tumor.

Characteristics

The true incidence of rhabdoid tumors in the population is not known due to the fact that many of these malignancies, especially in the brain, have been misdiagnosed. The most common sites of presentation for rhabdoid tumor are the kidney and brain. Rhabdoid tumors account for approximately 1–2 % of childhood renal tumors and 2 % of pediatric CNS tumors (Brain Tumor). Extrarenal tumors are less common and...

Keywords

Central Nervous System Tumor Primitive Neuroectodermal Tumor Rhabdoid Tumor Rhabdoid Cell Biallelic Inactivation 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.
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References

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Copyright information

© Springer-Verlag Berlin Heidelberg 2015

Authors and Affiliations

  1. 1.Children’s Hospital of Los AngelesLos AngelesUSA