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, SETD2, BAP1, TP53, and MTOR [2].

Most Commonly Altered Genes in Clear Cell Renal Cell Carcinoma

VHL Mutation, SETD2 Mutation, BAP1 Mutation, TP53 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

ARID1A +

ATM +

ATR +

ATRX +

BAP1 +

BARD1 +

BLM +

BRCA1 +

BRCA2 +

BRIP1 +

C11ORF30 +

CDK12 +

CDK2 +

CDK4 +

CHEK1 +

CHEK2 +

ERBB2 +

ERCC1 +

ERCC2 +

ERCC3 +

ERCC4 +

ERCC5 +

ERCC6 +

FAM175A +

FANCA +

FANCB +

FANCC +

FANCD2 +

FANCE +

FANCF +

FANCG +

FANCI +

FANCL +

FANCM +

FEN1 +

FH +

HDAC1 +

HDAC2 +

IDH1 +

IDH2 +

KRAS +

MCPH1 +

MDM2 +

MDM4 +

MLF1 +

MLH1 +

MLH3 +

MRE11A +

MSH2 +

MSH3 +

MSH6 +

MUTYH +

MYC +

NBN +

NPM1 +

PALB2 +

PARP1 +

PARP2 +

PMS1 +

PMS2 +

POLD1 +

POLE +

PPP2R1A +

PPP2R2A +

PRKDC +

PTEN +

RAD50 +

RAD51 +

RAD51B +

RAD51C +

RAD51D +

RAD52 +

RAD54L +

RB1 +

RPA1 +

SDHA +

SDHB +

SDHC +

SDHD +

SETD2 +

SLX4 +

SMARCB1 +

STAG2 +

STK11 +

TP53 +

WRN +

XRCC1 +

Disease Details

Synonyms
Renal cell carcinoma, clear cell adenocarcinoma, Hypernephroma, Conventional Renal Cell Carcinoma, Clear Cell Carcinoma of the Kidney, Clear Cell Carcinoma of Kidney, Renal Clear Cell Carcinoma, Clear Cell Adenocarcinoma, Kidney, Clear Cell Adenocarcinoma of Kidney, Conventional (Clear Cell) Renal Cell Adenocarcinoma, RCC, clear cell adenocarcinoma, Grawitz Tumor, Conventional (Clear Cell) Renal Cell Carcinoma, Kidney Clear Cell Adenocarcinoma, Kidney Clear Cell Carcinoma, Clear Cell Adenocarcinoma of the Kidney, Renal Clear Cell Adenocarcinoma
Parent(s)
Clear Cell Adenocarcinoma
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 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.