# Comprehensive molecular characterisation of papillary renal cell carcinoma (The Cancer Genome Atlas)

Source: https://onco.cc/key-papers/paper-tcga-papillary-rcc-nejm-2016/  
OnCo record `paper-tcga-papillary-rcc-nejm-2016` (Key paper). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

Genomic analysis of 161 papillary kidney cancers showed that type 1 tumours are driven by MET alterations while type 2 tumours are a mixture of distinct diseases including CDKN2A-silenced, SETD2-mutated, fumarate hydratase-deficient and a CpG island methylator phenotype with very poor survival.

## Summary

Integrated genomic study by The Cancer Genome Atlas of 161 papillary renal cell carcinomas showing MET alterations in 81 percent of type 1 tumours, and in type 2 tumours CDKN2A loss, SETD2 and other chromatin modifier mutations, TFE3 fusions, NRF2-ARE pathway activation and a CpG island methylator phenotype associated with fumarate hydratase deficiency and the worst outcomes.

## Fields

- Kind: Key paper
- Last checked: 2026-09-17
- Journal: New England Journal of Medicine
- Year: 2016
- DOI: 10.1056/NEJMoa1505917
- Authors: Cancer Genome Atlas Research Network, Linehan WM, Spellman PT, et al.
- Findings: MET alterations in 81 percent of type 1 tumours.; CpG island methylator phenotype subgroup with fumarate hydratase loss and poor survival among type 2 tumours.
- What it means: Papillary renal cell carcinoma is several diseases; the finding of MET dependence in type 1 tumours underpins the use of cabozantinib and savolitinib, and the 2022 WHO classification dropped the type 1 and 2 split in favour of molecular entities.
- Caveats: Treatment-naive primary tumours; metastatic disease may differ.

## Sources

- N Engl J Med 2016: https://doi.org/10.1056/NEJMoa1505917
- PubMed: https://pubmed.ncbi.nlm.nih.gov/26536169/

## Connected records

- cancers: [Papillary renal cell carcinoma](https://onco.cc/cancers/papillary-rcc/)
- journals: [New England Journal of Medicine](https://onco.cc/journals/nejm/)

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