{"entity":{"id":"chromophobe-rcc","kind":"cancer","name":"Chromophobe renal cell carcinoma","aka":["chRCC"],"tldr":"Chromophobe kidney cancer comes from a different cell of the kidney's tubules, usually behaves gently and is cured by surgery. Its rare metastatic form responds poorly to immunotherapy, so kinase and mTOR inhibitors are used, and it runs in families with Birt-Hogg-Dube syndrome.","summary":"Chromophobe renal cell carcinoma arises from the intercalated cells of the collecting duct and is marked by loss of whole chromosomes (1, 2, 6, 10, 13, 17, 21) with TP53 and PTEN mutations in a minority; it must be told apart from the benign oncocytoma, which it resembles. It is a feature of Birt-Hogg-Dube syndrome, caused by germline FLCN mutations, along with skin fibrofolliculomas and lung cysts. Most tumours are found early and cured by partial nephrectomy, and surveillance is reasonable for small lesions. Metastatic disease is uncommon, responds poorly to PD-1 blockade and is treated with sunitinib or cabozantinib, everolimus, or lenvatinib plus everolimus, drawing on the mTOR pathway activity seen in the disease; sarcomatoid transformation carries the worst prognosis of any kidney cancer.","asOf":"2026-09-17","wikipedia":"https://en.wikipedia.org/wiki/Chromophobe_renal_cell_carcinoma","links":[{"label":"Wikipedia","url":"https://en.wikipedia.org/wiki/Chromophobe_renal_cell_carcinoma"}],"tags":["subtype-page"],"related":[],"cancers":[],"sections":[],"technologies":[],"targets":[],"drugs":[],"companies":[],"institutions":[],"pathways":[],"terms":[],"trials":[],"people":[],"bottlenecks":[],"keyPapers":[],"journals":[],"notes":[],"group":"genitourinary","burden":"About five percent of kidney cancers, with the best outlook of the common types: fewer than one in ten spread, and those that do are often slow, but sarcomatoid change turns it deadly.","subtypes":["Classic chromophobe","Eosinophilic chromophobe","Birt-Hogg-Dube-associated (germline FLCN, hybrid oncocytic tumours)","Chromophobe with sarcomatoid change (aggressive)"],"biomarkers":["Multiple whole-chromosome losses","TP53 and PTEN mutations (poor outlook)","CK7 and KIT positive, distinguishing it from oncocytoma","Germline FLCN testing when syndromic features are present"],"standardOfCare":[{"setting":"Localised","approach":"Partial nephrectomy or ablation; active surveillance for small tumours; no adjuvant therapy.","refs":["thermal-ablation","active-surveillance"]},{"setting":"Metastatic","approach":"Sunitinib, cabozantinib, everolimus or lenvatinib plus everolimus; immunotherapy has low response rates outside sarcomatoid disease; trials preferred.","refs":["sunitinib","cabozantinib","everolimus","lenvatinib"]},{"setting":"Birt-Hogg-Dube syndrome","approach":"Kidney surveillance with MRI, nephron-sparing surgery at 3 cm, and genetic counselling.","refs":["rcc"]}],"stateOfArt":["Recognition of mTOR pathway dependence gave chromophobe cancer a rational medical option after immunotherapy disappointed.","Molecular tools now reliably separate chromophobe carcinoma from oncocytoma, sparing some patients surgery.","Registries of rare kidney cancers are producing the first subtype-specific outcome data."],"history":[{"year":1985,"title":"Thoenes describes chromophobe renal cell carcinoma","refs":[]},{"year":2002,"title":"FLCN identified as the Birt-Hogg-Dube gene","refs":[]},{"year":2014,"title":"TCGA maps chromosome losses and TP53 and PTEN mutations","refs":[]}],"pipeline":["lenvatinib","everolimus"],"openProblems":["No randomised trial has ever been run in chromophobe cancer.","Immunotherapy rarely works and the reason is not understood.","Sarcomatoid change has no effective treatment."],"parent":"rcc"},"route":"/cancers/chromophobe-rcc/","neighbours":{"drug":[{"id":"cabozantinib","kind":"drug","name":"Cabozantinib","route":"/drugs/cabozantinib/"},{"id":"everolimus","kind":"drug","name":"Everolimus","route":"/drugs/everolimus/"},{"id":"lenvatinib","kind":"drug","name":"Lenvatinib","route":"/drugs/lenvatinib/"},{"id":"sunitinib","kind":"drug","name":"Sunitinib","route":"/drugs/sunitinib/"}],"technology":[{"id":"active-surveillance","kind":"technology","name":"Active surveillance","route":"/technologies/active-surveillance/"},{"id":"thermal-ablation","kind":"technology","name":"Thermal ablation (RFA, microwave, cryo)","route":"/technologies/thermal-ablation/"}],"cancer":[{"id":"rcc","kind":"cancer","name":"Renal cell carcinoma","route":"/cancers/rcc/"}]}}