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Most cases of BHD syndrome\u2010associated RCC (BHD\u2010RCC) are less aggressive than sporadic clear cell RCC and multifocal. Therefore, it is critical to distinguish BHD\u2010RCC from its sporadic counterparts to identify and monitor affected families and to preserve renal function for as long as possible. The World Health Organization\/International Society of Urological Pathology consensus classification defined distinct entities for certain hereditary RCC; however, BHD\u2010RCC was not included in this classification. Although the clinical features and molecular mechanisms of BHD\u2010RCC have been investigated intensively over the last two decades, pathologists and urologists occasionally face difficulties in the diagnosis of BHD\u2010RCC that require genetic testing. Affected patients usually have miscellaneous benign disorders that often precede renal carcinogenesis. In the present review, we summarize the current understanding of the histopathological features of BHD\u2010RCC based on our epidemiological studies of Japanese families and a literature review. Pathological diagnostic clues and differential diagnosis of BHD\u2010RCC from other hereditary RCC are also briefly discussed.<\/jats:p>","DOI":"10.1111\/cas.14255","type":"journal-article","created":{"date-parts":[[2019,11,28]],"date-time":"2019-11-28T10:04:22Z","timestamp":1574935462000},"page":"15-22","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":33,"title":["Birt\u2010Hogg\u2010Dub\u00e9 syndrome\u2010associated renal cell carcinoma: Histopathological features and diagnostic conundrum"],"prefix":"10.1111","volume":"111","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6220-8179","authenticated-orcid":false,"given":"Mitsuko","family":"Furuya","sequence":"first","affiliation":[{"name":"Department of Molecular Pathology Yokohama City University Graduate School of Medicine Yokohama 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