Clear cell papillary renal cell carcinoma, renal angiomyoadenomatous tumor and renal cell carcinoma with leiomyomatous stroma-relationship of three types of renal tumors: A review

International audienceRenal angiomyoadenomatous tumor has been described in 2000, followed by description of clear cell papillary renal cell carcinoma in 2006. Discussion about possible relationship of both tumors were published since their description. As the main differencial diagnostic feature was considered presence/absence of fibroleiomyomatous stroma favoring diagnosis of RAT in stroma-rich tumors. However it was shown, that stroma is reactive, non-neoplastic by its nature and that all other histologic, immunohistochemical, and molecular-genetic features of both entities are identical. In upcomming WHO classification of renal tumors (2016) both lesions are considered as single entity (CCPRCC). Vaste majority of the published cases followed benign/indolent clinical course. Also vaste majority of the tumors has normal status of VHL gene (methylation, LOH 3p, mutations), however CCPRCC was referred in patients with VHL syndrome, Another issue covered by this review is possible relationship of CCPRCC and „renal cell carcinoma with leiomyomatous stroma“ (RCCLS). RCCLS shows clear cell cytology, abundand leiomyomatous stroma. Some of RCCLS are positive for CK 7, some negative. Similar situation exists for relation of RCCLS and VHL gene abnormalities. It is so far unclear, whether any relation between CCPRCC and RCCLS exists. From all published studies seems that these tumors are less likely each other relate