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We report a rare case of a primary PEComa of the liver and its characteristics on contrast-enhanced ultrasound (CEUS) in a 51-year-old female patient with an incidental finding of a hypoechoic liver lesion with peripheral hypervascularization on Doppler ultrasound. CEUS showed homogenous hypervascularity in the arterial phase that was consistent in the portal phase. In the late phase, a central washout phenomenon was evident. Histopathologic findings on sonographic biopsy of the lesion revealed a mesenchymal tumor with positivity for melanocytic markers Human Melanin Black-45 (HMB45) and Melan-A consistent with a PEComa. Despite the absence of high-risk features for malignancy, surgical resection was recommended due to the uncertain malignant potential of PEComas. The patient refused the operation and preferred sonographic follow-up; the lesion was stable over a period of 2\u00a0years. CEUS can provide valuable information regarding PEComa. After histological confirmation, the choice between resection and a watchful waiting must be made on individual basis.<\/jats:p>","DOI":"10.1007\/s12328-023-01779-w","type":"journal-article","created":{"date-parts":[[2023,3,25]],"date-time":"2023-03-25T10:02:32Z","timestamp":1679738552000},"page":"444-449","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":19,"title":["Hepatic perivascular epithelioid cell tumor (PEComa): contrast-enhanced ultrasound (CEUS) characteristics\u2014a case report and literature review"],"prefix":"10.1007","volume":"16","author":[{"given":"Sami","family":"Matrood","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christian","family":"G\u00f6rg","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ehsan","family":"Safai Zadeh","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1308-988X","authenticated-orcid":false,"given":"Amjad","family":"Alhyari","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2023,3,25]]},"reference":[{"key":"1779_CR1","first-page":"1","volume-title":"Pathology and genetics of tumours of soft tissue and bone","year":"2002","unstructured":"Fletcher CD, Unni KK, Mertens F, editors. 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