{"entity":{"id":"collecting-duct-carcinoma","kind":"cancer","name":"Collecting duct carcinoma of the kidney","aka":["Bellini duct carcinoma","Carcinoma of the collecting ducts of Bellini","Collecting duct renal cell carcinoma","Collecting duct and medullary (SMARCB1)"],"tldr":"Collecting duct carcinoma is a very rare, aggressive kidney cancer that starts in the tubes deep in the kidney that collect urine. It is usually found after it has spread, does not respond to the usual kidney cancer drugs, and is treated with the platinum chemotherapy used for bladder cancer, with surgery where possible.","summary":"Collecting duct carcinoma is a high-grade adenocarcinoma of the distal nephron, medullary in location, with tubular or tubulopapillary growth and a desmoplastic stroma; the 2022 WHO classification keeps it as a type and separates SMARCB1-deficient renal medullary carcinoma, its closest relative, as a molecularly defined type (Moch 2022). In a series of 39 collecting duct and 13 renal medullary carcinomas, both favoured the right kidney, centred on the medulla, averaged 7 cm, and showed poorly differentiated histology with desmoplasia, inflammation, lymphovascular invasion and perinephric extension in nearly all (Am J Surg Pathol 2012). In SEER, 74.4 percent presented at stage III or IV and most tumours were under 7 cm (Frontiers in Oncology 2021).\n\nHow it differs from its parent: renal cell carcinoma's VEGF and checkpoint pathways do not carry over; a systematic review found a 26 percent objective response to gemcitabine with cisplatin or carboplatin in 23 patients with metastatic disease and no response to immunotherapy in 49 patients treated in two older studies (Current Oncology 2013).\n\nHow common: 286 US cases in 15 years (Frontiers in Oncology 2021).\n\nTreatment: nephrectomy where the disease is resectable; gemcitabine with cisplatin or carboplatin for metastatic disease, borrowed from urothelial cancer, as the review describes; checkpoint inhibitor combinations are used off the renal cell carcinoma pathway without trial evidence in this type (Current Oncology 2013).","asOf":"2026-09-24","wikipedia":"https://en.wikipedia.org/wiki/Collecting_duct_carcinoma","links":[{"label":"NCI PDQ: renal cell cancer treatment","url":"https://www.cancer.gov/types/kidney/patient/kidney-treatment-pdq"},{"label":"Cancer Research UK: types and grades of kidney cancer","url":"https://www.cancerresearchuk.org/about-cancer/kidney-cancer/stages-types-grades/types-grades"},{"label":"Moch 2022, European Urology: the 2022 WHO classification of urinary and male genital tumours, part A (renal, penile, testicular)","url":"https://doi.org/10.1016/j.eururo.2022.06.016"},{"label":"Frontiers in Oncology 2021: collecting duct carcinoma of the kidney, 286 SEER patients 2004 to 2018","url":"https://doi.org/10.3389/fonc.2021.727222"},{"label":"Current Oncology 2013: management of renal collecting duct carcinoma, systematic review","url":"https://doi.org/10.3747/co.20.1230"},{"label":"Am J Surg Pathol 2012: collecting duct carcinoma and renal medullary carcinoma, 52 cases","url":"https://doi.org/10.1097/pas.0b013e3182635954"}],"tags":["subtype-page","wave4","kidney","rare"],"related":["rcc","renal-medullary-carcinoma","urothelial","papillary-rcc","fh-deficient-renal-cell-carcinoma","tfe3-rearranged-renal-cell-carcinoma"],"cancers":[],"sections":[],"technologies":[],"targets":[],"drugs":["gemcitabine-cisplatin","gemcitabine","cisplatin","carboplatin"],"companies":[],"institutions":[],"pathways":[],"terms":["nephrectomy"],"trials":[],"people":[],"bottlenecks":[],"keyPapers":[],"journals":[],"dependsOn":[],"notes":[],"group":"genitourinary","burden":"Very rare and falling: 286 patients in the US SEER registry over 2004 to 2018, median age 59, 67.5 percent men, 74.4 percent stage III or IV at diagnosis (Frontiers in Oncology 2021). Cancer Research UK lists clear cell at 70 to 80 percent, papillary 5 to 10 and chromophobe 3 to 5 percent of renal cell cancers; collecting duct carcinoma is well under 1 percent.","subtypes":["Collecting duct carcinoma, classic (medullary epicentre, desmoplastic; right kidney predilection)","Collecting duct carcinoma with sarcomatoid change","SMARCB1-deficient renal medullary carcinoma (own page; formerly grouped here)"],"biomarkers":["Retained SMARCB1 (INI1) expression, distinguishing it from renal medullary carcinoma","High nuclear grade and lymphovascular invasion","Stage at diagnosis (three quarters III or IV)"],"standardOfCare":[{"setting":"All stages","approach":"Nephrectomy where resectable; gemcitabine with cisplatin or carboplatin for metastatic disease, borrowed from urothelial cancer; renal cell carcinoma drugs have little evidence here.","refs":["nephrectomy","gemcitabine-cisplatin","carboplatin","rcc"]}],"stateOfArt":[],"history":[],"pipeline":[],"openProblems":[],"parent":"rcc"},"route":"/cancers/collecting-duct-carcinoma/","neighbours":{"cancer":[{"id":"urothelial","kind":"cancer","name":"Bladder & urothelial cancer","route":"/cancers/urothelial/"},{"id":"fh-deficient-renal-cell-carcinoma","kind":"cancer","name":"Fumarate hydratase-deficient renal cell carcinoma (HLRCC-associated)","route":"/cancers/fh-deficient-renal-cell-carcinoma/"},{"id":"papillary-rcc","kind":"cancer","name":"Papillary renal cell carcinoma","route":"/cancers/papillary-rcc/"},{"id":"rcc","kind":"cancer","name":"Renal cell carcinoma","route":"/cancers/rcc/"},{"id":"renal-medullary-carcinoma","kind":"cancer","name":"Renal medullary carcinoma (SMARCB1-deficient)","route":"/cancers/renal-medullary-carcinoma/"},{"id":"tfe3-rearranged-renal-cell-carcinoma","kind":"cancer","name":"TFE3-rearranged (translocation) renal cell carcinoma","route":"/cancers/tfe3-rearranged-renal-cell-carcinoma/"}],"drug":[{"id":"carboplatin","kind":"drug","name":"Carboplatin","route":"/drugs/carboplatin/"},{"id":"cisplatin","kind":"drug","name":"Cisplatin","route":"/drugs/cisplatin/"},{"id":"gemcitabine","kind":"drug","name":"Gemcitabine","route":"/drugs/gemcitabine/"},{"id":"gemcitabine-cisplatin","kind":"drug","name":"Gemcitabine + cisplatin","route":"/drugs/gemcitabine-cisplatin/"}],"term":[{"id":"nephrectomy","kind":"term","name":"Nephrectomy","route":"/terms/nephrectomy/"}]}}