{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,26]],"date-time":"2026-07-26T04:22:20Z","timestamp":1785039740925,"version":"3.55.0"},"reference-count":23,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2024,4,30]],"date-time":"2024-04-30T00:00:00Z","timestamp":1714435200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2024,4,30]],"date-time":"2024-04-30T00:00:00Z","timestamp":1714435200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["World J Surg Onc"],"abstract":"<jats:title>Abstract<\/jats:title><jats:sec>\n                <jats:title>Purpose<\/jats:title>\n                <jats:p>The objective of this paper is to offer a thorough examination of the clinical presentations, etiology, and treatment strategies associated with perivascular epithelioid cell tumors (PEComas).<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Methods<\/jats:title>\n                <jats:p>This retrospective study examined the comprehensive archival data of PEComa cases diagnosed at Beijing Hospital from 2015 to 2023. The pathology slides of all patients were thoroughly reassessed by two experienced pathologists. A thorough retrospective analysis was undertaken, incorporating clinicopathological data including gender, age at diagnosis, initial clinical manifestations, signs, disease onset site, tumor markers, imaging findings, therapeutic modalities, pathological features, immunohistochemical profiles, treatment responses, and prognostic indicators. Patients were evaluated for disease severity according to established pathological classification criteria and were followed up until the designated analysis cut-off date. In instances where patients were unable to be monitored on-site, they were contacted via telephone for postoperative follow-up inquiries.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Results<\/jats:title>\n                <jats:p>This study included 11 patients with ages ranging from 17 to 66 years old, presenting with the disease in multiple anatomical sites, including the retroperitoneum (2\/11), liver (4\/11), kidney (4\/11), lung (1\/11), and broad ligament of the uterus (1\/11). Most patients presented with non-specific clinical symptoms and were subsequently diagnosed with space-occupying lesions upon physical examination. The tumor demonstrated progressive growth and enlargement, which could result in compression of neighboring organs. Preoperative imaging alone is insufficient for a definitive diagnosis of PEComa, but MRI can provide an initial evaluation of the tumor\u2019s potential malignancy. Molecular marker testing specific to PEComa, such as HMB-45 (90.0%), SMA (81.8%), Melan-A (90.9%), vimentin (90.9%), and Desmin (36.3%), was conducted on all patients. No adjuvant therapies were administered postoperatively. Upon analysis, no instances of relapse at the primary site or the development of new tumors at other sites were observed. Regular imaging reviews of three patients with malignant PEComa post-surgery showed no evidence of recurrence.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Conclusions<\/jats:title>\n                <jats:p>The clinical presentation, tumor biomarkers, and imaging characteristics of PEComa lack specificity, necessitating dependence on pathology and immunohistochemistry for precise diagnosis. The mainstay of treatment consists of surgical resection, with patients typically experiencing a favorable prognosis.<\/jats:p>\n              <\/jats:sec>","DOI":"10.1186\/s12957-024-03349-x","type":"journal-article","created":{"date-parts":[[2024,4,30]],"date-time":"2024-04-30T08:02:47Z","timestamp":1714464167000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["A retrospective clinical analysis of 11 cases of PEComa from different sites"],"prefix":"10.1186","volume":"22","author":[{"given":"Jinbowen","family":"Yan","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dan","family":"Zhou","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yifei","family":"Wang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bowen","family":"Yang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yuefeng","family":"Wang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kaili","family":"Zhang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shuo","family":"Zhang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bo","family":"Zhang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Qingwei","family":"Meng","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Qiubo","family":"Lv","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2024,4,30]]},"reference":[{"issue":"8","key":"3349_CR1","doi-asserted-by":"publisher","first-page":"667","DOI":"10.1016\/j.prp.2016.03.010","volume":"212","author":"E Kudela","year":"2016","unstructured":"Kudela E, Biringer K, Kasajova P, Nachajova M, Adamkov M. Perivascular epithelioid cell tumors of the uterine cervix. Pathol Res Pract. 2016;212(8):667\u201371.","journal-title":"Pathol Res Pract"},{"issue":"5","key":"3349_CR2","doi-asserted-by":"publisher","first-page":"359","DOI":"10.1016\/j.anndiagpath.2015.06.003","volume":"19","author":"K Thway","year":"2015","unstructured":"Thway K, Fisher C. PEComa: morphology and genetics of a complex tumor family. Annals Diagn Pathol. 2015;19(5):359\u201368.","journal-title":"Annals Diagn Pathol"},{"key":"3349_CR3","doi-asserted-by":"publisher","first-page":"121","DOI":"10.1016\/j.ejro.2018.08.004","volume":"5","author":"R De\u017eman","year":"2018","unstructured":"De\u017eman R, Ma\u0161ulovi\u0107 D, Popovi\u010d P. Hepatic perivascular epithelioid cell tumor: a case report. Eur J Radiol open. 2018;5:121\u20135.","journal-title":"Eur J Radiol open"},{"key":"3349_CR4","doi-asserted-by":"publisher","first-page":"193","DOI":"10.1016\/j.ijscr.2018.10.063","volume":"53","author":"PV Voulgari","year":"2018","unstructured":"Voulgari PV, Tatsi V, Milionis HJ, Goussia A, Xydis V, Glantzounis GK. Liver perivascular epithelioid cell tumor in a patient with systemic lupus erythematosus. Int J Surg case Rep. 2018;53:193\u20135.","journal-title":"Int J Surg case Rep"},{"key":"3349_CR5","doi-asserted-by":"publisher","first-page":"1783","DOI":"10.2147\/OTT.S300523","volume":"14","author":"J Gu","year":"2021","unstructured":"Gu J, Wang W, Wang S. A retrospective case study of 13 uterine Perivascular Epithelioid Cell Neoplasm (PEComa) patients. OncoTargets Therapy. 2021;14:1783\u201390.","journal-title":"OncoTargets Therapy"},{"key":"3349_CR6","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, et al. Perivascular epithelioid cell neoplasm (PEComa) of the uterus: a systematic review. Int J Surg (London England). 2015;19:1\u20135.","journal-title":"Int J Surg (London England)"},{"issue":"1","key":"3349_CR7","doi-asserted-by":"publisher","first-page":"129","DOI":"10.1016\/j.pathol.2020.08.008","volume":"53","author":"A Cali\u00f2","year":"2021","unstructured":"Cali\u00f2 A, Brunelli M, Segala D, Zamboni G, Bonetti F, Pea M, et al. Angiomyolipoma of the kidney: from simple hamartoma to complex tumour. Pathology. 2021;53(1):129\u201340.","journal-title":"Pathology"},{"issue":"19","key":"3349_CR8","doi-asserted-by":"publisher","first-page":"2299","DOI":"10.3748\/wjg.v27.i19.2299","volume":"27","author":"P Calame","year":"2021","unstructured":"Calame P, Tyrode G, Weil Verhoeven D, F\u00e9lix S, Klompenhouwer AJ, Di Martino V, et al. Clinical characteristics and outcomes of patients with hepatic angiomyolipoma: a literature review. World J Gastroenterol. 2021;27(19):2299\u2013311.","journal-title":"World J Gastroenterol"},{"issue":"3","key":"3349_CR9","doi-asserted-by":"publisher","first-page":"238","DOI":"10.1016\/j.pan.2018.02.007","volume":"18","author":"M Zizzo","year":"2018","unstructured":"Zizzo M, Ugoletti L, Tumiati D, Castro Ruiz C, Bonacini S, Panebianco M, et al. Primary pancreatic perivascular epithelioid cell tumor (PEComa): a surgical enigma. A systematic review of the literature. Pancreatology: Official J Int Association Pancreatology (IAP) [et al]. 2018;18(3):238\u201345.","journal-title":"Pancreatology: Official J Int Association Pancreatology (IAP) [et al]"},{"issue":"28","key":"3349_CR10","doi-asserted-by":"publisher","first-page":"e3890","DOI":"10.1097\/MD.0000000000003890","volume":"95","author":"Z Chen","year":"2016","unstructured":"Chen Z, Han S, Wu J, Xiong M, Huang Y, Chen J, et al. A systematic review: perivascular epithelioid cell tumor of gastrointestinal tract. Medicine. 2016;95(28):e3890.","journal-title":"Medicine"},{"key":"3349_CR11","doi-asserted-by":"publisher","first-page":"60","DOI":"10.1186\/1746-1596-8-60","volume":"8","author":"X Fu","year":"2013","unstructured":"Fu X, Jiang JH, Gu X, Li Z. Malignant perivascular epithelioid cell tumor of mesentery with lymph node involvement: a case report and review of literature. Diagn Pathol. 2013;8:60.","journal-title":"Diagn Pathol"},{"issue":"1","key":"3349_CR12","doi-asserted-by":"publisher","first-page":"80","DOI":"10.3350\/cmh.2016.0034","volume":"23","author":"HJ Son","year":"2017","unstructured":"Son HJ, Kang DW, Kim JH, Han HY, Lee MK. Hepatic perivascular epithelioid cell tumor (PEComa): a case report with a review of literatures. Clin Mol Hepatol. 2017;23(1):80\u20136.","journal-title":"Clin Mol Hepatol"},{"issue":"2","key":"3349_CR13","doi-asserted-by":"publisher","first-page":"176","DOI":"10.1097\/PAS.0000000000000133","volume":"38","author":"JK Schoolmeester","year":"2014","unstructured":"Schoolmeester JK, Howitt BE, Hirsch MS, Dal Cin P, Quade BJ, Nucci MR. Perivascular epithelioid cell neoplasm (PEComa) of the gynecologic tract: clinicopathologic and immunohistochemical characterization of 16 cases. Am J Surg Pathol. 2014;38(2):176\u201388.","journal-title":"Am J Surg Pathol"},{"issue":"12","key":"3349_CR14","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":"10","key":"3349_CR15","doi-asserted-by":"publisher","first-page":"1395","DOI":"10.1097\/PAS.0b013e3181f17ac0","volume":"34","author":"P Argani","year":"2010","unstructured":"Argani P, Aulmann S, Illei PB, Netto GJ, Ro J, Cho HY, et al. A distinctive subset of PEComas harbors TFE3 gene fusions. Am J Surg Pathol. 2010;34(10):1395\u2013406.","journal-title":"Am J Surg Pathol"},{"issue":"1","key":"3349_CR16","doi-asserted-by":"publisher","first-page":"62","DOI":"10.1186\/s12957-021-02462-5","volume":"20","author":"R Purwar","year":"2022","unstructured":"Purwar R, Soni K, Shukla M, Verma A, Kumar T, Pandey M. TFE3-associated perivascular epithelioid cell tumor with complete response to mTOR inhibitor therapy: report of first case and literature review. World J Surg Oncol. 2022;20(1):62.","journal-title":"World J Surg Oncol"},{"issue":"5","key":"3349_CR17","doi-asserted-by":"publisher","first-page":"607","DOI":"10.1007\/s00428-014-1655-x","volume":"465","author":"Q Shen","year":"2014","unstructured":"Shen Q, Rao Q, Xia QY, Yu B, Shi QL, Zhang RS, et al. Perivascular epithelioid cell tumor (PEComa) with TFE3 gene rearrangement: clinicopathological, immunohistochemical, and molecular features. Virchows Archiv: Int J Pathol. 2014;465(5):607\u201313.","journal-title":"Virchows Archiv: Int J Pathol"},{"key":"3349_CR18","doi-asserted-by":"publisher","first-page":"582087","DOI":"10.3389\/fonc.2020.582087","volume":"10","author":"J Xu","year":"2020","unstructured":"Xu J, Gong XL, Wu H, Zhao L. Case Report: gastrointestinal PEComa with TFE3 rearrangement treated with Anti-VEGFR TKI apatinib. Front Oncol. 2020;10:582087.","journal-title":"Front Oncol"},{"issue":"2","key":"3349_CR19","doi-asserted-by":"publisher","first-page":"258","DOI":"10.1016\/j.pathol.2022.12.340","volume":"55","author":"JS Saleh","year":"2023","unstructured":"Saleh JS, Whittington CP, Bresler SC, Patel RM. Mesenchymal tumours with melanocytic expression: a potential pitfall in the differential diagnosis of malignant melanoma. Pathology. 2023;55(2):258\u201368.","journal-title":"Pathology"},{"issue":"4","key":"3349_CR20","doi-asserted-by":"publisher","first-page":"555","DOI":"10.1016\/j.pathol.2022.08.011","volume":"55","author":"E Paver","year":"2023","unstructured":"Paver E, Aivazian K, Maclean F, Long GV, Spillane AJ, Wilmott JS, et al. Utility of next generation sequencing for differentiating malignant PEComa from metastatic melanoma. Pathology. 2023;55(4):555\u201360.","journal-title":"Pathology"},{"issue":"1","key":"3349_CR21","doi-asserted-by":"publisher","first-page":"7","DOI":"10.1186\/s40001-017-0248-y","volume":"22","author":"BS Kwon","year":"2017","unstructured":"Kwon BS, Suh DS, Lee NK, Song YJ, Choi KU, Kim KH. Two cases of perivascular epithelioid cell tumor of the uterus: clinical, radiological and pathological diagnostic challenge. Eur J Med Res. 2017;22(1):7.","journal-title":"Eur J Med Res"},{"issue":"2","key":"3349_CR22","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. Medicine. 2019;98(2):e14072.","journal-title":"Medicine"},{"issue":"33","key":"3349_CR23","doi-asserted-by":"publisher","first-page":"3660","DOI":"10.1200\/JCO.21.01728","volume":"39","author":"AJ Wagner","year":"2021","unstructured":"Wagner AJ, Ravi V, Riedel RF, Ganjoo K, Van Tine BA, Chugh R, et al. Nab-Sirolimus for patients with malignant perivascular epithelioid cell tumors. J Clin Oncology: Official J Am Soc Clin Oncol. 2021;39(33):3660\u201370.","journal-title":"J Clin Oncology: Official J Am Soc Clin Oncol"}],"container-title":["World Journal of Surgical Oncology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s12957-024-03349-x.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1186\/s12957-024-03349-x\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s12957-024-03349-x.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,4,30]],"date-time":"2024-04-30T08:06:41Z","timestamp":1714464401000},"score":1,"resource":{"primary":{"URL":"https:\/\/wjso.biomedcentral.com\/articles\/10.1186\/s12957-024-03349-x"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,4,30]]},"references-count":23,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2024,12]]}},"alternative-id":["3349"],"URL":"https:\/\/doi.org\/10.1186\/s12957-024-03349-x","relation":{},"ISSN":["1477-7819"],"issn-type":[{"value":"1477-7819","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,4,30]]},"assertion":[{"value":"10 August 2023","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"24 February 2024","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"30 April 2024","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"All procedures performed in this study were in accordance with the ethical standards of the institutional and\/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards. For this type of study, a formal consent was not required.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethical approval"}},{"value":"The authors declare no competing interests.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"116"}}