{"entity":{"id":"hereditary-papillary-renal-cell-carcinoma","kind":"term","name":"Hereditary papillary renal cell carcinoma","aka":["HPRC","HPRCC","hereditary papillary renal carcinoma"],"tldr":"Hereditary papillary renal cell carcinoma is a rare inherited condition, caused by an activating change in the MET gene, that carries a very high lifetime risk of papillary kidney cancer. It is managed by kidney specialists with experience of inherited kidney cancer.","summary":"Autosomal dominant, caused by activating germline variants in the MET proto-oncogene (the hepatocyte growth factor receptor). The NCI PDQ summary of kidney cancer genetics lists papillary renal cell carcinoma (formerly type 1) as the tumour, a cumulative cancer risk approaching 100%, and no associated non-renal tumours. Clinical genetic tests for MET are available in CLIA-certified laboratories, and genetic counselling is a prerequisite.\n\nNo management guideline specific to this syndrome was read in this round: the PDQ summary gives no surveillance schedule, so none is recorded.","asOf":"2026-11-04","links":[{"label":"NCI PDQ: genetics of kidney cancer (health professional version)","url":"https://www.cancer.gov/types/kidney/hp/kidney-genetics-pdq"},{"label":"Orphanet ORPHA:47044","url":"https://www.orpha.net/en/disease/detail/47044"}],"tags":["gap-fill","hereditary"],"related":["birt-hogg-dube-syndrome","vhl-disease"],"cancers":["papillary-rcc"],"sections":[],"technologies":["germline-testing"],"targets":["met"],"drugs":[],"companies":[],"institutions":[],"pathways":[],"terms":["hereditary-cancer-syndromes"],"trials":[],"people":[],"bottlenecks":[],"keyPapers":[],"journals":[],"dependsOn":[],"notes":["Orphanet ORPHA:47044. In the NHS directory it is tested under R224, inherited renal cancer.","Open gap: a primary source for surveillance and nephron-sparing management in HPRC."],"category":"Genomics & genetics"},"route":"/terms/hereditary-papillary-renal-cell-carcinoma/","neighbours":{"term":[{"id":"birt-hogg-dube-syndrome","kind":"term","name":"Birt-Hogg-Dubé syndrome","route":"/terms/birt-hogg-dube-syndrome/"},{"id":"hereditary-cancer-syndromes","kind":"term","name":"Hereditary cancer syndromes","route":"/terms/hereditary-cancer-syndromes/"},{"id":"vhl-disease","kind":"term","name":"Von Hippel-Lindau disease","route":"/terms/vhl-disease/"}],"cancer":[{"id":"papillary-rcc","kind":"cancer","name":"Papillary renal cell carcinoma","route":"/cancers/papillary-rcc/"}],"technology":[{"id":"germline-testing","kind":"technology","name":"Germline (hereditary) testing","route":"/technologies/germline-testing/"}],"target":[{"id":"met","kind":"target","name":"MET","route":"/targets/met/"}]}}