{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,8]],"date-time":"2026-10-08T10:00:38Z","timestamp":1791453638214,"version":"4.3.3"},"reference-count":25,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2022,12,16]],"date-time":"2022-12-16T00:00:00Z","timestamp":1671148800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2022,12,16]],"date-time":"2022-12-16T00:00:00Z","timestamp":1671148800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"name":"Beijing Kanghua Foundation for the Develpoment of Traditional Chinese and Western Medicine","award":["KH-2021-LLZX-070"],"award-info":[{"award-number":["KH-2021-LLZX-070"]}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["BMC Women's Health"],"abstract":"<jats:title>Abstract<\/jats:title><jats:sec>\n                <jats:title>Background<\/jats:title>\n                <jats:p>Perivascular epithelioid cell tumors (PEComas) of the uterus is a rare type of mesenchymal tumors associated with myelomelanocytic differentiation and distinctive histological appearances. So far, the reported cases of uterine PEComas are usually benign. Documented malignant cases with aggressive behavior appear to be less common.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Case presentation<\/jats:title>\n                <jats:p>We report a 37-year-old female who received abdominal hysterectomy for uterine tumor in a local hospital. She was diagnosed with uterine leiomyosarcoma and referred to Hubei Cancer Hospital. Her histological slides were reviewed and immunohistochemical staining for specific markers of epithelial, melanocytic, myoid and some others were analyzed. The pathologic diagnosis was malignant uterine PEComa. Systematic imaging of the patient further revealed an abdominal para-aortic mass. She received pelvic and para-aortic lymph node dissection. Postoperative histology revealed para-aortic lymph nodal metastasis of malignant uterine PEComa. She received 8 cycles of chemotherapy after surgery. The chemotherapy regiment was epirubicin plus ifosfamide The patient is free of recurrence and metastasis 6 years after surgical resection.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Conclusion<\/jats:title>\n                <jats:p>Uterine PEComas are indistinguishable from other uterine tumors such as leiomyoma and leiomyosarcoma before pathologic diagnosis could be made. For patients with malignant uterine PEComas, removal of both primary lesions and metastatic foci, if any, needs to be attempted. Postoperative chemotherapy or radiotherapy should also be considered in patients with distant metastases or positive lymph nodes.<\/jats:p>\n              <\/jats:sec>","DOI":"10.1186\/s12905-022-02119-9","type":"journal-article","created":{"date-parts":[[2022,12,16]],"date-time":"2022-12-16T03:03:04Z","timestamp":1671159784000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":11,"title":["Malignant perivascular epithelioid cell tumor (PEComa) of the uterus"],"prefix":"10.1186","volume":"22","author":[{"given":"Bo","family":"Cao","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7608-4843","authenticated-orcid":false,"given":"Yi","family":"Huang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2022,12,16]]},"reference":[{"issue":"6","key":"2119_CR1","doi-asserted-by":"publisher","first-page":"722","DOI":"10.1097\/00000478-199606000-00010","volume":"20","author":"G Zamboni","year":"1996","unstructured":"Zamboni G, Pea M, Martignoni G, Zancanaro C, Faccioli G, Gilioli E, Pederzoli P, Bonetti F. Clear cell \u201csugar\u201d tumor of the pancreas. A novel member of the family of lesions characterized by the presence of perivascular epithelioid cells. Am J Surg Pathol. 1996;20(6):722\u201330.","journal-title":"Am J Surg Pathol"},{"key":"2119_CR2","first-page":"221","volume-title":"World Health Organization classification of tumors: pathology and genetics of tumors of soft tissue and bone","author":"A Folpe","year":"2002","unstructured":"Folpe A. Neoplasms with perivascular epithelioid cell differentiation (PEComas). In: Fletcher C, Unni K, Mertens F, editors. World Health Organization classification of tumors: pathology and genetics of tumors of soft tissue and bone. Lyon: IARC Press; 2002. p. 221\u20132."},{"issue":"12","key":"2119_CR3","doi-asserted-by":"publisher","first-page":"1558","DOI":"10.1097\/01.pas.0000173232.22117.37","volume":"29","author":"AL Folpe","year":"2005","unstructured":"Folpe AL, Mentzel T, Lehr HA, Fisher C, Balzer BL, Weiss SW. Perivascular epithelioid cell neoplasms of soft tissue and gynecologic origin: a clinicopathologic study of 26 cases and review of the literature. Am J Surg Pathol. 2005;29(12):1558\u201375.","journal-title":"Am J Surg Pathol"},{"issue":"2","key":"2119_CR4","doi-asserted-by":"publisher","first-page":"e14072","DOI":"10.1097\/MD.0000000000014072","volume":"98","author":"CH Liu","year":"2019","unstructured":"Liu CH, Chao WT, Lin SC, Lau HY, Wu HH, Wang PH. Malignant perivascular epithelioid cell tumor in the female genital tract: Preferred reporting items for systematic reviews and meta-analyses. Med (Baltim). 2019;98(2):e14072.","journal-title":"Med (Baltim)"},{"issue":"12","key":"2119_CR5","doi-asserted-by":"publisher","first-page":"681","DOI":"10.1097\/01.mph.0000193475.06870.d5","volume":"27","author":"IS Jeon","year":"2005","unstructured":"Jeon IS, Lee SM. Multimodal treatment using surgery, radiotherapy, and chemotherapy in a patient with a perivascular epithelioid cell tumor of the uterus. J Pediatr Hematol Oncol. 2005;27(12):681\u20134.","journal-title":"J Pediatr Hematol Oncol"},{"issue":"1","key":"2119_CR6","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1097\/00000478-200201000-00001","volume":"26","author":"R Vang","year":"2002","unstructured":"Vang R, Kempson RL. Perivascular epithelioid cell tumor (\u2018PEComa\u2019) of the uterus: a subset of HMB-45-positive epithelioid mesenchymal neoplasms with an uncertain relationship to pure smooth muscle tumors. Am J Surg Pathol. 2002;26(1):1\u201313.","journal-title":"Am J Surg Pathol"},{"key":"2119_CR7","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1016\/j.ijsu.2015.05.002","volume":"19","author":"A Musella","year":"2015","unstructured":"Musella A, De Felice F, Kyriacou AK, Barletta F, Di Matteo FM, Marchetti C, Izzo L, Monti M, Benedetti Panici P, Redler A, et al. 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J Clin Pathol. 2003;56(8):627\u20138.","journal-title":"J Clin Pathol"}],"container-title":["BMC Women's Health"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s12905-022-02119-9.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1186\/s12905-022-02119-9\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s12905-022-02119-9.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,12,16]],"date-time":"2022-12-16T03:03:14Z","timestamp":1671159794000},"score":1,"resource":{"primary":{"URL":"https:\/\/bmcwomenshealth.biomedcentral.com\/articles\/10.1186\/s12905-022-02119-9"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,12,16]]},"references-count":25,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2022,12]]}},"alternative-id":["2119"],"URL":"https:\/\/doi.org\/10.1186\/s12905-022-02119-9","relation":{},"ISSN":["1472-6874"],"issn-type":[{"value":"1472-6874","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,12,16]]},"assertion":[{"value":"7 April 2021","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"6 December 2022","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"16 December 2022","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"Ethics committee approval was obtained from Hubei Cancer Hospital (No.LLHBCH2022YN-011). Written informed consent was obtained from the patient.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"Personal or clinical details along with any identifying images to be published in this study was obtained from the patient and Hubei Cancer Hospital. We obtained the patient\u2019s written consent and she signed a written informed consent.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"The authors declare that they have no conflict of interest.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"523"}}