Renal Cell Carcinoma (RCC)

Overview

Renal Cell Carcinoma (RCC) is the umbrella OncoTree node for primary carcinomas arising from the renal parenchyma. Its major OncoTree children include Renal Clear Cell Carcinoma (CCRCC), Renal Non-Clear Cell Carcinoma (NCCRCC), and Unclassified Renal Cell Carcinoma (URCC). NCCRCC itself encompasses Papillary Renal Cell Carcinoma (PRCC), Chromophobe Renal Cell Carcinoma (CHRCC), and Translocation-Associated Renal Cell Carcinoma (TRCC) as well as URCC. URCC comprises approximately 4–5% of all RCC and lacks an established standard of care PMID:27713405.

Cohorts in the corpus

• urcc_mskcc_2016 — 62 high-grade primary unclassified RCC tumors from MSKCC, MSK-IMPACT 230-gene targeted sequencing, RNA-seq, OncoScan SNP arrays, FISH, and IHC PMID:27713405.

See also cohorts listed under subtype pages: CCRCC, PRCC, CHRCC, TRCC.

Recurrent alterations

Alterations vary substantially by RCC subtype. Cross-subtype observations from the corpus:

• VHL — driver in ~75% of CCRCC but only 1/62 (1.6%) of URCC, underscoring molecular divergence across RCC subtypes PMID:27713405.
• NF2, MTOR, TSC1, TSC2, FH — recurrently altered in URCC; mTOR-pathway mutations also frequent in CHRCC (23%) and relevant in CCRCC PMID:27713405.
• SETD2, BAP1 — shared chromatin-modifier drivers across CCRCC, PRCC, and URCC PMID:27713405.

See individual subtype pages for full alteration catalogs. - PIPseq cohort: VHL V166G mutation identified in a renal cell carcinoma patient as diagnostic of Von Hippel-Lindau syndrome PMID:28007021

Subtypes

• Renal Clear Cell Carcinoma (CCRCC) — most common RCC subtype; VHL/3p loss-driven.
• Renal Non-Clear Cell Carcinoma (NCCRCC) — umbrella for non-clear-cell subtypes.
  • Papillary Renal Cell Carcinoma (PRCC)
  • Chromophobe Renal Cell Carcinoma (CHRCC)
  • Translocation-Associated Renal Cell Carcinoma (TRCC)
  • Unclassified Renal Cell Carcinoma (URCC)

Therapeutic landscape

See individual subtype pages. Notable pan-RCC observations from the corpus:

• mTOR-pathway alterations (MTOR, TSC1/2, PTEN) occur across CCRCC, CHRCC, and URCC, providing a shared rationale for mTOR-inhibitor evaluation in subsets of each subtype PMID:27713405.
• Molecularly defined subsets of URCC (NF2-loss, mTORC1-hyperactive, FH-deficient, ALK-fusion) support subset-specific therapy approaches rather than a pan-URCC standard PMID:27713405.

Sources

• PMID:27713405 — Chen et al., molecular characterization of 62 MSKCC high-grade uRCCs; defines molecular subtypes of URCC and contextualizes within broader RCC nosology.

This page was processed by crosslinker on 2026-05-14. - PMID:28007021

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