Overview

NCI Definition: A rhabdoid tumor which arises in the soft tissues. It occurs in infants and children and may be associated with loss of chromosome 22. It is characterized by the presence of cells with a large eccentric nucleus, prominent nucleolus, and abundant cytoplasm. [1]

Significant Genes in Extrarenal Rhabdoid Tumor

MDM2 +

MDM4 +

PPM1D +

SMARCA4 +

SMARCB1 +

TET2 +

Disease Details

Synonyms
Rhabdoid Tumor of Soft Tissue, Malignant Extrarenal Rhabdoid Neoplasm
Parent(s)
Rhabdoid Tumor
Children
Extrarenal Rhabdoid Tumor of the Liver

References

1. National Cancer Institute. NCI Thesaurus Version 18.11d. https://ncit.nci.nih.gov/ncitbrowser/ [2018-08-28]. [2018-09-21].

2. The AACR Project GENIE Consortium. AACR Project GENIE: powering precision medicine through an international consortium. Cancer Discovery. 2017;7(8):818-831. Dataset Version 6. This dataset does not represent the totality of the genetic landscape; see paper for more information.

3. All assertions and clinical trial landscape data are curated from primary sources. You can read more about the curation process here.