Abstract
Renal cell carcinomas (RCCs) is a group of various malignant tumours of the renal cortex displaying distinct clinical, morphologic, and genetic features. Clear cell papillary renal cell carcinoma (ccpRCC), belonging to this group, shares morphologic features with both clear cell renal cell carcinoma (ccRCC) and papillary renal cell carcinoma (pRCC) and therefore, more strict diagnostic criteria should be developed to avoid misdiagnosis. Despite overlapping features, ccpRCC has also distinct clinical behaviour, histologic characteristics (morphologic and immunohistochemical), and genomic features. The concepts concerning this tumour are constantly developing since its biological potential and molecular basis remains to be fully unravelled. First reports indicated the presence of ccpRCC in end-stage renal disease, and they underlined the enriched development in this group of patients; however, currently, it is known that such tumours can also occur spontaneously in the normal kidney. Numerous studies have demonstrated that clinical outcomes and prognosis of ccpRCC patients is highly favourable. Till now, no convincing evidence of metastatic ccpRCC or death caused by the disease has been found. Therefore, it is of high importance to correctly differentiate ccpRCC from other subtypes of RCC with a much worse prognosis and to introduce appropriate management.
Highlights
First reports indicated the presence of Clear cell papillary renal cell carcinoma (ccpRCC) in end-stage renal disease (ESRD), and they underlined the enriched development in this group of patients; currently, it is known that such tumours can occur spontaneously in the normal kidney and even a greater their prevalence has been described in non-end-stage renal disease patients [6,8]
It has been found that in general ccpRCC are small and well encapsulated mix of branched glandular structures, cystic, papillary, tubular/acinar structures, solid sheet-like or nested components closely imitating clear cell renal cell carcinoma, all submerged in clear cytoplasm [6,8,15]
Cystic changes are the predominant growth pattern in ccpRCC; such formations may be suggestive of multilocular cystic clear-cell Renal cell carcinomas (RCCs), specific nuclear arrangements accompanied by at least focal papillary architecture are indicative of ccpRCC
Summary
Publisher’s Note: MDPI stays neutral with regard to jurisdictional claims in published maps and institutional affiliations. The International Society of Urological Pathology (ISUP) Vancouver Classification of renal neoplasia uses the diagnostic term “clear cell (tubulo) papillary renal cell carcinoma (ccpRCC)” [4,5] The concepts concerning this tumour are constantly developing since its biological potential and molecular basis remain to be fully unravelled. The prevalence of ccpRCC is estimated to range from 1 to 4.3%; this is the fourth most common subtype of renal cell carcinoma, preceded only by clear cell, papillary, and chromophobe renal cell carcinomas [6,7] This type of tumour occurs generally at the age of 18–88 years with a mean of 70 years, and there is no gender predilection [4,5]. Some scientists even postulate the reclassification of this entity as a benign or low malignant potential neoplasm due to its aforementioned non-aggressive nature and the resemblance to several extrarenal benign neoplasms [12,13]
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