{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,23]],"date-time":"2026-10-23T00:07:32Z","timestamp":1792714052788,"version":"4.5.0"},"reference-count":39,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2017,4,12]],"date-time":"2017-04-12T00:00:00Z","timestamp":1491955200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100008456","name":"A. Alfred Taubman Medical Research Institute","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100008456","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100008732","name":"Uehara Memorial Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100008732","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Histopathology"],"published-print":{"date-parts":[[2017,7]]},"abstract":"<jats:sec><jats:title>Aims<\/jats:title><jats:p>Fumarate hydratase (<jats:styled-content style=\"fixed-case\">FH<\/jats:styled-content>)\u2010deficient renal cell carcinoma (<jats:styled-content style=\"fixed-case\">RCC<\/jats:styled-content>) is a high\u2010grade, aggressive tubulopapillary carcinoma, arising predominantly in the setting of the hereditary leiomyomatosis\u2013<jats:styled-content style=\"fixed-case\">RCC<\/jats:styled-content> syndrome of familial uterocutaneous leiomyomatosis and deficiency of <jats:styled-content style=\"fixed-case\">FH<\/jats:styled-content>. In contrast, succinate dehydrogenase (<jats:styled-content style=\"fixed-case\">SDH<\/jats:styled-content>)\u2010deficient <jats:styled-content style=\"fixed-case\">RCC<\/jats:styled-content> is a lower\u2010grade oncocytic carcinoma with cytoplasmic flocculence\/vacuolation and inclusions, arising mostly in individuals harbouring germline mutations of subunit B of the <jats:styled-content style=\"fixed-case\">SDH<\/jats:styled-content> complex (<jats:styled-content style=\"fixed-case\">SDHB<\/jats:styled-content>). Herein we aim to report the clinicopathologic features of a novel form of <jats:styled-content style=\"fixed-case\">FH<\/jats:styled-content>\u2010deficient <jats:styled-content style=\"fixed-case\">RCC<\/jats:styled-content> showing a low grade oncocytic morphology, reminiscent of <jats:styled-content style=\"fixed-case\">SDH<\/jats:styled-content>\u2010deficient <jats:styled-content style=\"fixed-case\">RCC<\/jats:styled-content>.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods and results<\/jats:title><jats:p>These distinctive, low\u2010grade oncocytic neoplasms, with solid, nested and focally tubular architecture (2\u201390 mm), arose in four males (aged 11\u201341 years). Uniform cytology of polygonal cells, with flocculent, vacuolated eosinophilic cytoplasm with scattered inclusions, fine chromatin, and inconspicuous nucleoli, was apparent. Despite these features suggestive of <jats:styled-content style=\"fixed-case\">SDH<\/jats:styled-content>\u2010deficient <jats:styled-content style=\"fixed-case\">RCC<\/jats:styled-content>, each tumour was confirmed as an <jats:styled-content style=\"fixed-case\">FH<\/jats:styled-content>\u2010deficient carcinoma with retained <jats:styled-content style=\"fixed-case\">SDHB<\/jats:styled-content> expression. One case showed a synchronous, anatomically separate, typical high\u2010grade <jats:styled-content style=\"fixed-case\">FH<\/jats:styled-content>\u2010deficient <jats:styled-content style=\"fixed-case\">RCC<\/jats:styled-content>; one other showed such a tumour at nephrectomy 4 years later. No progression has been noted at 3 and 7 years in the cases with only the <jats:styled-content style=\"fixed-case\">SDH<\/jats:styled-content>\u2010like lesions; the two cases with separate, typical <jats:styled-content style=\"fixed-case\">FH<\/jats:styled-content>\u2010deficient <jats:styled-content style=\"fixed-case\">RCC<\/jats:styled-content>s progressed.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>In summary, we characterize a novel oncocytic type of <jats:styled-content style=\"fixed-case\">FH<\/jats:styled-content>\u2010deficient <jats:styled-content style=\"fixed-case\">RCC<\/jats:styled-content> with a striking resemblance to <jats:styled-content style=\"fixed-case\">SDH<\/jats:styled-content>\u2010deficient <jats:styled-content style=\"fixed-case\">RCC<\/jats:styled-content>, posing a diagnostic challenge and raising concerns about sampling and multifocality for syndrome\u2010associated cases under surveillance protocols.<\/jats:p><\/jats:sec>","DOI":"10.1111\/his.13183","type":"journal-article","created":{"date-parts":[[2017,2,6]],"date-time":"2017-02-06T14:16:52Z","timestamp":1486390612000},"page":"42-52","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":111,"title":["A distinctive, low\u2010grade oncocytic fumarate hydratase\u2010deficient renal cell carcinoma, morphologically reminiscent of succinate dehydrogenase\u2010deficient renal cell carcinoma"],"prefix":"10.1111","volume":"71","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0982-4607","authenticated-orcid":false,"given":"Steven C","family":"Smith","sequence":"first","affiliation":[{"name":"Departments of Pathology and Urology VCU School of Medicine Richmond VA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Deepika","family":"Sirohi","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine Cedars\u2010Sinai 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