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As they morphologically and immunohistochemically resemble smooth muscle tumors, distinction between the two entities is often challenging, and may be aided by molecular analysis. Thus far, two distinct molecular groups\u2010classic PEComas with <jats:italic>TSC<\/jats:italic> mutations and <jats:italic>TFE3<\/jats:italic>\u2010translocation associated PEComas with <jats:italic>TFE3<\/jats:italic> fusions have been described. Recognition of the first group is imperative as these patients may benefit from targeted therapy with mTOR inhibitors, if malignant. This review will focus on recognition of the morphologic and immunophenotypic features of PEComas, as well as the role of molecular testing in their diagnosis and treatment, analysis of the different algorithms to predict behavior, and differential diagnosis.<\/jats:p>","DOI":"10.1002\/gcc.22908","type":"journal-article","created":{"date-parts":[[2020,10,25]],"date-time":"2020-10-25T06:54:45Z","timestamp":1603608885000},"page":"168-179","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":50,"title":["Perivascular epithelioid cell tumors (<scp>PEComa<\/scp>) of the gynecologic tract"],"prefix":"10.1002","volume":"60","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8155-7131","authenticated-orcid":false,"given":"Jennifer A.","family":"Bennett","sequence":"first","affiliation":[{"name":"Department of Pathology University of Chicago Medicine  Chicago Illinois 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