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Heterozygous germline mutations in <jats:italic>FH<\/jats:italic> result in hereditary leiomyomatosis and renal cell carcinoma, a cancer predisposition syndrome. <jats:italic>FH<\/jats:italic> deficiency has multiple oncogenic mechanisms including through promotion of aerobic glycolysis, induction of pseudohypoxia, post-translational protein modification and impairment of DNA damage repair by homologous recombination. FH-deficient neoplasms can present with characteristic morphological features that raise suspicion for <jats:italic>FH<\/jats:italic> alterations and also frequently demonstrate loss of FH immunoreactivity and intracellular accumulation of 2-succinocysteine, also detected by immunohistochemistry.<\/jats:p>","DOI":"10.1136\/jclinpath-2021-207830","type":"journal-article","created":{"date-parts":[[2021,8,5]],"date-time":"2021-08-05T13:27:48Z","timestamp":1628170068000},"page":"615-619","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":30,"title":["Gene of the month: FH"],"prefix":"10.1136","volume":"74","author":[{"given":"Roman E","family":"Zyla","sequence":"first","affiliation":[{"name":"Laboratory Medicine and Pathobiology, University of Toronto, Toronto, Ontario, 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