Overview

NCI Definition: A malignant epithelial neoplasm of the kidney characterized by the presence of lipid-containing clear cells within a vascular network. The tumor may metastasize to unusual sites and late metastasis is common. [1]

Clear cell renal cell carcinomas most frequently harbor alterations in VHL, PBRM1, SETD2, BAP1, and KDM5C [2].

Most Commonly Altered Genes in Clear Cell Renal Cell Carcinoma

VHL Mutation, PBRM1fs, PBRM1 Mutation, SETD2 Mutation, and MTOR Mutation are the most common alterations in clear cell renal cell carcinoma [2].

Top Alterations in Clear Cell Renal Cell Carcinoma

Significant Genes in Clear Cell Renal Cell Carcinoma

ATM +

ATR +

BARD1 +

BRCA1 +

BRCA2 +

BRIP1 +

CDK12 +

CHEK1 +

CHEK2 +

FANCA +

FANCB +

FANCC +

FANCD2 +

FANCE +

FANCF +

FANCG +

FANCI +

FANCL +

FANCM +

FH +

IDH1 +

IDH2 +

KRAS +

MCPH1 +

MRE11A +

MYC +

NBN +

PALB2 +

PTEN +

RAD50 +

RAD51 +

RAD51B +

RAD51C +

RAD51D +

RAD54L +

RB1 +

SDHA +

SDHB +

SDHC +

SDHD +

SETD2 +

SLX4 +

TP53 +

Disease Details

Synonyms
Grawitz Tumor, Clear Cell Adenocarcinoma of the Kidney, Hypernephroma, Conventional (Clear Cell) Renal Cell Carcinoma, Clear Cell Adenocarcinoma of Kidney, RCC, clear cell adenocarcinoma, Clear Cell Carcinoma of the Kidney, Kidney Clear Cell Carcinoma, Conventional (Clear Cell) Renal Cell Adenocarcinoma, Clear Cell Carcinoma of Kidney, Conventional Renal Cell Carcinoma, Renal Clear Cell Adenocarcinoma, Kidney Clear Cell Adenocarcinoma, Renal cell carcinoma, clear cell adenocarcinoma, Renal Clear Cell Carcinoma, Clear Cell Adenocarcinoma, Kidney
Parent(s)
Renal Cell Carcinoma
Children
Clear Cell Papillary Renal Cell Carcinoma and Renal Clear Cell Carcinoma with Sarcomatoid Features
OncoTree Name
Renal Clear Cell Carcinoma
OncoTree Code
CCRCC

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 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.