Papillary Renal Cell Carcinoma (PRCC)

Overview

Papillary Renal Cell Carcinoma (PRCC) is a subtype of non-clear cell renal cell carcinoma (nccRCC) originating in the kidney tubular epithelium, characterised by papillary or tubular architecture. It sits under NCCRCC in OncoTree (tissue: Kidney) and is the most common non-clear cell RCC subtype.

Cohorts in the corpus

Recurrent alterations

  • Integrated exome/RNA-seq/CNV profiling of 67 pRCCs identified ten significantly mutated genes: MET (15%, including novel activating kinase-domain mutations), NF2, SLC5A3, PNKD, CPQ, LRP2, CHD3, NHERF1, SETD2, and CRTC1; ~70% of pRCCs harbour chr7 amplification encompassing MET; mutational burden in pRCC exceeded ccRCC (mean 61 vs 45 protein-coding alterations, P=3.6×10⁻⁷) PMID:25401301.
  • A novel transforming ACTG1-MITF gene fusion was identified in one pRCC sample, activating HIF1A, MET, and APEX1 targets PMID:25401301.
  • URCC (n=62, MSKCC) harbors MET H1094Y (T62) and BAP1/SETD2 chromatin alterations overlapping PRCC biology; NF2/SETD2-mutant cases are noted to be histologically near the type 2 pRCC boundary PMID:27713405.

Subtypes

  • MiTF-high pRCC — characterised by MITF/TFE3/TFEB fusions, amplifications, or overexpression; BIRC7 is overexpressed and proposed as a therapeutic target PMID:25401301.
  • A five-gene RNA-seq classifier (ASB1, GLYAT, PDZK1IP1, PLCG2, SDCBP2) separates pRCC from chRCC and renal oncocytoma at 95.3% accuracy PMID:25401301.

Therapeutic landscape

  • MET-targeted therapy: MET mutations (15%) and chr7/MET amplification (~70%) support stratified trials of MET inhibitors in pRCC PMID:25401301.

Sources

  • PMID:25401301 — Durinck et al., integrated genomic profiling of 167 non-clear cell RCCs including 67 pRCCs.

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