{"entity":{"id":"paper-tcga-papillary-rcc-nejm-2016","kind":"paper","name":"Comprehensive molecular characterisation of papillary renal cell carcinoma (The Cancer Genome Atlas)","aka":[],"tldr":"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.","asOf":"2026-09-17","links":[{"label":"N Engl J Med 2016","url":"https://doi.org/10.1056/NEJMoa1505917"},{"label":"PubMed","url":"https://pubmed.ncbi.nlm.nih.gov/26536169/"}],"tags":[],"related":[],"cancers":["papillary-rcc"],"sections":[],"technologies":[],"targets":[],"drugs":[],"companies":[],"institutions":[],"pathways":[],"terms":[],"trials":[],"people":[],"bottlenecks":[],"keyPapers":[],"journals":["nejm"],"dependsOn":[],"notes":[],"journal":"New England Journal of Medicine","year":2016,"doi":"10.1056/NEJMoa1505917","pmid":"26536169","authors":"Cancer Genome Atlas Research Network, Linehan WM, Spellman PT, et al.","paperType":"translational","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."],"whatItMeans":"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."],"changedPractice":true,"participants":161},"route":"/key-papers/paper-tcga-papillary-rcc-nejm-2016/","neighbours":{"cancer":[{"id":"papillary-rcc","kind":"cancer","name":"Papillary renal cell carcinoma","route":"/cancers/papillary-rcc/"}],"journal":[{"id":"nejm","kind":"journal","name":"New England Journal of Medicine","route":"/journals/nejm/"}]}}