{"entity":{"id":"clear-cell-papillary-renal-cell-tumour","kind":"cancer","name":"Clear cell papillary renal cell tumour","aka":["Clear cell papillary renal cell carcinoma","Clear cell papillary renal cell tumour (indolent, separate in WHO 2022)","Clear cell tubulopapillary renal cell carcinoma","Renal angiomyoadenomatous tumour (overlapping term)"],"tldr":"Clear cell papillary renal cell tumour is a small, low-grade kidney tumour that looks like a mix of clear cell and papillary kidney cancer but behaves harmlessly: no case has been reported to spread. The WHO renamed it from carcinoma to tumour in 2022 for that reason. It is common in people with kidney failure and is cured by removing it.","summary":"The 2016 WHO classification recognised clear cell papillary renal cell carcinoma as a type, and the 2022 edition renamed it clear cell papillary renal cell tumour because no metastasis has been documented (Moch 2016; Moch 2022). It is composed of clear cells lining cystic, tubular and papillary structures with nuclei aligned away from the basement membrane, is strongly CK7-positive with cup-shaped carbonic anhydrase IX staining, and lacks the VHL alterations and 3p loss of clear cell carcinoma and the trisomies of papillary carcinoma. In 55 tumours from 34 patients (8 with end-stage renal disease), tumours were 0.2 to 7.5 cm (mean 2.0), 87 percent grade 2 and 96 percent stage pT1a, multiple in 9 patients (Modern Pathology 2013). In 290 consecutive nephrectomies it was the fourth most common type, average age 58, a third associated with end-stage renal disease, all pT1, and two cases had been misclassified as clear cell carcinoma (Human Pathology 2014).\n\nHow it differs from its parent: it is the renal cell tumour that is not, in behaviour, a cancer; misclassifying it as clear cell carcinoma can lead to over-treatment and to unnecessary VHL testing.\n\nHow common: 3 to 4 percent of renal tumours coming to surgery (Modern Pathology 2013; Human Pathology 2014).\n\nTreatment: partial nephrectomy or, where appropriate, active surveillance of a small mass; no systemic therapy is needed and none has been studied.","asOf":"2026-09-24","wikipedia":"https://en.wikipedia.org/wiki/Renal_cell_carcinoma","links":[{"label":"Moch 2016, European Urology: the 2016 WHO classification of urinary and male genital tumours, part A","url":"https://doi.org/10.1016/j.eururo.2016.02.029"},{"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":"Modern Pathology 2013: clear cell papillary renal cell carcinoma, 55 tumours, differential diagnosis and immunoprofile","url":"https://doi.org/10.1038/modpathol.2012.204"},{"label":"Human Pathology 2014: clear cell papillary RCC is the fourth most common type in 290 nephrectomies","url":"https://doi.org/10.1016/j.humpath.2013.08.004"},{"label":"Cancer Research UK: types and grades of kidney cancer","url":"https://www.cancerresearchuk.org/about-cancer/kidney-cancer/stages-types-grades/types-grades"}],"tags":["subtype-page","wave4","kidney","rare"],"related":["rcc","clear-cell-rcc","papillary-rcc","mucinous-tubular-spindle-cell-carcinoma"],"cancers":[],"sections":[],"technologies":[],"targets":[],"drugs":[],"companies":[],"institutions":[],"pathways":[],"terms":["nephrectomy"],"trials":[],"people":[],"bottlenecks":[],"keyPapers":[],"journals":[],"dependsOn":[],"notes":[],"group":"genitourinary","burden":"3 percent of adult renal cell carcinoma resections over three years at one centre (Modern Pathology 2013) and 4.1 percent of 290 consecutive nephrectomies for renal cell carcinoma, the fourth most common type after clear cell (70 percent), papillary (16.6) and chromophobe (5.9) (Human Pathology 2014).","subtypes":["Sporadic clear cell papillary renal cell tumour (solitary, pT1a)","Clear cell papillary renal cell tumour in end-stage renal disease (often multiple)","Renal angiomyoadenomatous tumour (smooth muscle-rich variant)"],"biomarkers":["Strong CK7 and cup-shaped carbonic anhydrase IX staining; CD10 and AMACR negative","No VHL mutation or 3p loss","Low grade (WHO/ISUP 1 or 2) and pT1 stage","End-stage renal disease"],"standardOfCare":[{"setting":"All cases","approach":"Partial nephrectomy or active surveillance of a small mass; no systemic therapy.","refs":["rcc","nephrectomy","clear-cell-rcc"]}],"stateOfArt":[],"history":[],"pipeline":[],"openProblems":[],"parent":"rcc"},"route":"/cancers/clear-cell-papillary-renal-cell-tumour/","neighbours":{"cancer":[{"id":"clear-cell-rcc","kind":"cancer","name":"Clear cell renal cell carcinoma","route":"/cancers/clear-cell-rcc/"},{"id":"mucinous-tubular-spindle-cell-carcinoma","kind":"cancer","name":"Mucinous tubular and spindle cell carcinoma of the kidney","route":"/cancers/mucinous-tubular-spindle-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/"}],"term":[{"id":"nephrectomy","kind":"term","name":"Nephrectomy","route":"/terms/nephrectomy/"}]}}