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]

Rhabdoid tumor of the kidneys most frequently harbor alterations in SMARCB1, SPEN, RB1, RASA1, and ARID1B [2].

Most Commonly Altered Genes in Rhabdoid Tumor of the Kidney

SMARCB1 Mutation, SMARCB1 Loss, SPEN L945fs, SMARCB1fs, and SMARCB1 R201* are the most common alterations in rhabdoid tumor of the kidney [2].

Top Alterations in Rhabdoid Tumor of the Kidney

Significant Genes in Rhabdoid Tumor of the Kidney

MDM2 +

MDM4 +




TET2 +

Disease Details

Rhabdoid Tumour of Kidney, Malignant Rhabdoid Tumor of Kidney, Kidney Rhabdoid Tumor, Rhabdoid Tumour of the Kidney, Rhabdoid Neoplasm of Kidney, Renal Rhabdoid Tumor, Renal Rhabdoid Neoplasm, Rhabdoid Tumor of the Kidney (RTK), MRTK, Malignant Rhabdoid Tumor of the Kidney, Rhabdoid Neoplasm of the Kidney
Rhabdoid Tumor
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 8. 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.