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Ultrasonographic virtual organ computer-aided analysis showed the mass completely formed by arteriovenous vessels, and that allowed distinction from leiomyosarcoma. Repeated haemoperitoneum required uterine artery embolisation. Mass revascularisation occurred in the following 7\u2009days. A laparotomic hysterectomy with removal of the uterus and right parametrium was performed in epidural analgesia. Histological features were consistent with the diagnosis of uterine PEComa of uncertain malignant features, in the presence of coexisting pulmonary LAM. In women with LAM, acute haemoperitoneum may indicate the presence of a uterine PEComa whose diagnosis can be challenging.<\/jats:p>","DOI":"10.1136\/bcr-2021-244257","type":"journal-article","created":{"date-parts":[[2021,11,2]],"date-time":"2021-11-02T13:20:20Z","timestamp":1635859220000},"page":"e244257","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":3,"title":["Acute and repeated haemoperitoneum: a challenging case of lymphangioleiomyomatosis with uterine PEComa"],"prefix":"10.1136","volume":"14","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8887-3823","authenticated-orcid":false,"given":"Anjeza","family":"Xholli","sequence":"first","affiliation":[{"name":"DINOGMI, Obsterics and Gynecology Clinic, IRCCS Ospedale San Martino, Genova, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chiara","family":"Kratochwila","sequence":"additional","affiliation":[{"name":"Obstetrics and Gynecology Clinic, IRCCS AOU San Martino, Genova, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Valerio Gaetano","family":"Vellone","sequence":"additional","affiliation":[{"name":"Department of Surgical and Diagnostic Sciences, IRCCS Ospedale San Martino, Genova, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria Giulia","family":"Schiaffino","sequence":"additional","affiliation":[{"name":"Obstetrics and Gynecology Clinic, IRCCS AOU San Martino, Genova, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"239","published-online":{"date-parts":[[2021,11,2]]},"reference":[{"key":"2025082723372368000_14.11.e244257.1","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0b013e318235edbd"},{"key":"2025082723372368000_14.11.e244257.2","unstructured":"Fletcher CDM , Unni KK , Mertens F . 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