NCI Definition: A high grade carcinoma of the kidney. It is not a distinct clinicopathological entity and includes a diverse group of renal cell carcinomas which have been transformed from a lower to a higher grade. [1]

Sarcomatoid renal cell carcinomas most frequently harbor alterations in TP53, VHL, PTEN, CDKN2B, and CDKN2A [2].

Most Commonly Altered Genes in Sarcomatoid Renal Cell Carcinoma

VHL Mutation, TP53 Mutation, TP53 c.217-c.1178 Missense, TP53 Missense, and TP53 Exon 5 Mutation are the most common alterations in sarcomatoid renal cell carcinoma [2].

Top Alterations in Sarcomatoid Renal Cell Carcinoma

Significant Genes in Sarcomatoid Renal Cell Carcinoma


TFE3 +

TSC1 +

Disease Details

RCC w/ sarcomatoid features, Renal cell carcinoma with sarcomatoid features
Sarcomatoid Carcinoma
OncoTree Name
Sarcomatoid Renal Cell Carcinoma
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.