NCI Definition: A rhabdoid tumor that arises from the kidney. It occurs in children and it is associated with abnormalities of chromosome 22. It is characterized by the presence of cells with a large eccentric nucleus, prominent nucleolus, and abundant cytoplasm. The prognosis is poor. [1]

Significant Genes in Rhabdoid Tumor of the Kidney

MDM2 +

MDM4 +




TET2 +

Disease Details

Rhabdoid Tumour of the Kidney, Kidney Rhabdoid Tumor, Malignant Rhabdoid Tumor of Kidney, Renal Rhabdoid Neoplasm, MRTK, Rhabdoid Neoplasm of the Kidney, Rhabdoid Tumor of the Kidney (RTK), Rhabdoid Neoplasm of Kidney, Malignant Rhabdoid Tumor of the Kidney, Renal Rhabdoid Tumor, Rhabdoid Tumour of Kidney
Kidney Cancer
OncoTree Name
Rhabdoid Cancer
OncoTree Code


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.