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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.
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).
How 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.
How common: 3 to 4 percent of renal tumours coming to surgery (Modern Pathology 2013; Human Pathology 2014).
Treatment: partial nephrectomy or, where appropriate, active surveillance of a small mass; no systemic therapy is needed and none has been studied.
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).
Renal cell carcinoma comes from the kidney's filtering cortex, urothelial cancer from the lining of the collecting system and bladder, and the adrenal on top hosts cortical and medullary (neuroblastoma) tumours.
Same organ: Collecting duct carcinoma of the kidney, Renal medullary carcinoma (SMARCB1-deficient), TFE3-rearranged (translocation) renal cell carcinoma, Fumarate hydratase-deficient renal cell carcinoma (HLRCC-associated), Succinate dehydrogenase-deficient renal cell carcinoma, Mucinous tubular and spindle cell carcinoma of the kidney, Eosinophilic solid and cystic renal cell carcinoma, Urothelial carcinoma of the urethra, Squamous cell carcinoma of the urethra, Adenocarcinoma of the urethra (including clear cell adenocarcinoma), Melanoma of the urethra, Non-muscle-invasive bladder cancer, Muscle-invasive and advanced bladder cancer, Bladder & urothelial cancer, Clear cell renal cell carcinoma, Papillary renal cell carcinoma, Chromophobe renal cell carcinoma, Renal cell carcinoma, Wilms tumour (nephroblastoma), Neuroblastoma (paediatric), Low-risk neuroblastoma (INRG very low and low risk, including stage MS), Intermediate-risk neuroblastoma, High-risk neuroblastoma, Adrenocortical carcinoma, Pheochromocytoma and paraganglioma (PPGL), Urethral cancer, Penile cancer, Localised penile cancer (organ-confined, node-negative), Node-positive and metastatic penile cancer, Localised adrenocortical carcinoma (ENSAT stage I to III, resectable), Advanced and metastatic adrenocortical carcinoma (ENSAT stage IV or unresectable), Hereditary pheochromocytoma and paraganglioma (SDHx, VHL, RET, NF1, MAX and TMEM127), Metastatic pheochromocytoma and paraganglioma
Partial nephrectomy or active surveillance of a small mass; no systemic therapy.
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Query for this cancer: (TITLE:"Clear cell papillary renal cell tumour" OR ABSTRACT:"Clear cell papillary renal cell tumour" OR TITLE:"Clear cell papillary renal cell carcinoma" OR ABSTRACT:"Clear cell papillary renal cell carcinoma" OR TITLE:"Clear cell papillary renal cell tumour indolent, separate in WHO 2022" OR ABSTRACT:"Clear cell papillary renal cell tumour indolent, separate in WHO 2022" OR TITLE:"Clear cell tubulopapillary renal cell carcinoma" OR ABSTRACT:"Clear cell tubulopapillary renal cell carcinoma" OR TITLE:"Renal angiomyoadenomatous tumour overlapping term" OR ABSTRACT:"Renal angiomyoadenomatous tumour overlapping term") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Clear cell papillary renal cell tumour, not a curated reading list.
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