{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,8]],"date-time":"2026-10-08T04:34:32Z","timestamp":1791434072638,"version":"4.3.3"},"reference-count":29,"publisher":"BMJ","issue":"11","content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["BMJ Case Rep"],"accepted":{"date-parts":[[2022,10,25]]},"published-print":{"date-parts":[[2022,11]]},"abstract":"<jats:p>Perivascular epithelioid cell neoplasm (PEComa) is a rare type of tumour, and primary retroperitoneal PEComa is rarer still. Although pulmonary lymphangioleiomyomatosis (LAM), angiomyolipomas and clear cell \u2018sugar\u2019 tumours of the lung are well described, relatively little is known about other members of the PEComa family. We describe a case of an asymptomatic retroperitoneal PEComa, lymphangioleiomyoma type, which appeared in a previously healthy middle-aged woman as an incidental finding, in a CT scan performed in the context of spontaneous pneumothorax. The patient underwent surgical excision of the tumour and the histopathological and immunohistochemical analysis of the surgical specimen made the definitive diagnosis. Although rare, reports of isolated retroperitoneal lymphangioleiomyoma and primary retroperitoneal PEComas NOS (not otherwise specified) are described in the literature, normally associated with pulmonary LAM. The patient\u2019s pulmonary imaging was normal. Short-term re-examination did not detect any recurrence. We also provide a literature review of this rare group of tumours.<\/jats:p>","DOI":"10.1136\/bcr-2022-250466","type":"journal-article","created":{"date-parts":[[2022,11,11]],"date-time":"2022-11-11T10:00:17Z","timestamp":1668160817000},"page":"e250466","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":4,"title":["Primary retroperitoneal PEComa: an incidental finding"],"prefix":"10.1136","volume":"15","author":[{"given":"B\u00e1rbara Monteiro","family":"Marinho","sequence":"first","affiliation":[{"name":"Department of Surgery, HEBIPA - Hepatobiliary and Pancreatic Unit, Hospital de Santo Ant\u00f3nio, Centro Hospitalar Universit\u00e1rio do Porto, Porto, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ant\u00f3nio G\u00e2mboa","family":"Canha","sequence":"additional","affiliation":[{"name":"Department of Surgery, HEBIPA - Hepatobiliary and Pancreatic Unit, Hospital de Santo Ant\u00f3nio, Centro 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