{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,11]],"date-time":"2026-05-11T05:07:49Z","timestamp":1778476069237,"version":"3.51.4"},"reference-count":49,"publisher":"MDPI AG","issue":"9","license":[{"start":{"date-parts":[[2026,4,27]],"date-time":"2026-04-27T00:00:00Z","timestamp":1777248000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Cancers"],"abstract":"<jats:p>Background\/Objectives: Malignant PEComa is a rare sarcoma subtype and usually represents PEComa-NOS (not otherwise specified), one of the several entities of the PEComa family. Surgery is the primary treatment for localized disease; chemotherapy is used mainly for metastatic or unresectable cases. Radiotherapy (RT) may be considered in selected cases; however, its role remains unclear due to the rarity of the disease and limited radiotherapy-specific studies. Methods: This is a descriptive literature review of a limited number of reports on RT use in PEComa. Descriptive statistics were used to summarize reported case characteristics and outcomes. Results: We identified 28 publications reporting 33 cases. In neoadjuvant settings, there was a significant local response to RT in one case. In other neoadjuvant cases, although quantitative response assessments were not reported, most showed no recurrence during follow-up, with the longest follow-up at 34 months, suggesting that a possible benefit in local disease control may exist. In the adjuvant setting, some reports described prolonged disease-free survival following RT, though the lack of direct comparisons between surgery with versus without RT and heterogeneous follow-up periods limit definitive conclusions. In selected metastatic cases, palliative RT achieved notable local responses, potentially contributing to durable local control. Conclusions: In conclusion, although the only available data on RT in PEComas come from case studies with overall heterogeneous management approaches, RT has shown some potential as a therapeutic option across neoadjuvant, adjuvant, and palliative settings, warranting further dedicated clinical studies.<\/jats:p>","DOI":"10.3390\/cancers18091388","type":"journal-article","created":{"date-parts":[[2026,4,27]],"date-time":"2026-04-27T19:24:51Z","timestamp":1777317891000},"page":"1388","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Management of PEComas: A Review of the Role of Radiotherapy"],"prefix":"10.3390","volume":"18","author":[{"given":"Kristina","family":"Nesterova","sequence":"first","affiliation":[{"name":"Arthur J.E. Child Comprehensive Cancer Centre, Department of Medical Oncology, Calgary, AB T2N 5G2, Canada"},{"name":"University of Calgary, Calgary, AB T2N 1N4, Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Reinhardt","family":"Krcek","sequence":"additional","affiliation":[{"name":"Department of Radiation Oncology, University Hospital Zurich, University of Zurich, 8091 Zurich, Switzerland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0825-5070","authenticated-orcid":false,"given":"Abha A.","family":"Gupta","sequence":"additional","affiliation":[{"name":"University of Toronto, Toronto, ON M5S 3H2, Canada"},{"name":"Division of Hematology\/Oncology, Hospital for Sick Children, Toronto, ON M5G 1X8, Canada"},{"name":"Division of Medical Oncology, Princess Margaret Cancer Centre, University Health Network, Toronto, ON M5G 2M9, Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3815-7703","authenticated-orcid":false,"given":"Peter W. M.","family":"Chung","sequence":"additional","affiliation":[{"name":"University of Toronto, Toronto, ON M5S 3H2, Canada"},{"name":"Radiation Medicine Program, Princess Margaret Cancer Centre, University Health Network, Toronto, ON M5G 2M9, Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2026,4,27]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"2934","DOI":"10.1002\/cncr.33618","article-title":"Ultra-rare sarcomas: A consensus paper from the Connective Tissue Oncology Society community of experts on the incidence threshold and the list of entities","volume":"127","author":"Stacchiotti","year":"2021","journal-title":"Cancer"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"307","DOI":"10.1097\/00000478-199203000-00013","article-title":"PEC and sugar","volume":"16","author":"Bonetti","year":"1992","journal-title":"Am. J. Surg. Pathol."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"722","DOI":"10.1097\/00000478-199606000-00010","article-title":"Clear Cell \u201cSugar\u201d Tumor of the Pancreas: A Novel Member of the Family of Lesions Characterized by the Presence of Perivascular Epithelioid Cells","volume":"20","author":"Zamboni","year":"1996","journal-title":"Am. J. Surg. Pathol."},{"key":"ref_4","unstructured":"Fletcher, C.D.M., Unni, K., and Mertens, F. (2002). Pathology and Genetics of Tumours of Soft Tissue and Bone, IARC Press. [3rd ed.]. World Health Organization Classification of Tumours."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"1558","DOI":"10.1097\/01.pas.0000173232.22117.37","article-title":"Perivascular epithelioid cell neoplasms of soft tissue and gynecologic origin: A clinicopathologic study of 26 cases and review of the literature","volume":"29","author":"Folpe","year":"2005","journal-title":"Am. J. Surg. Pathol."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"102455","DOI":"10.1016\/j.ctrv.2022.102455","article-title":"Retrospective observational studies in ultra-rare sarcomas: A consensus paper from the Connective Tissue Oncology Society (CTOS) community of experts on the minimum requirements for the evaluation of activity of systemic treatments","volume":"110","author":"Stacchiotti","year":"2022","journal-title":"Cancer Treat. Rev."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"527","DOI":"10.1016\/j.jmig.2009.04.017","article-title":"Perivascular epithelioid cell neoplasms: A systematic review of prognostic factors","volume":"16","author":"Zekry","year":"2009","journal-title":"J. Minim. Invasive Gynecol."},{"key":"ref_8","first-page":"e65","article-title":"PEComa, a Rare Epithelioid Cell Tumor","volume":"93","year":"2015","journal-title":"Cir. Esp. (Engl. Ed.)"},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"11005","DOI":"10.1200\/JCO.2019.37.15_suppl.11005","article-title":"ABI-009 (nab-sirolimus) in advanced malignant perivascular epithelioid cell tumors (PEComa): Preliminary efficacy, safety, and mutational status from AMPECT, an open label phase II registration trial","volume":"37","author":"Wagner","year":"2019","journal-title":"J. Clin. Oncol."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"3660","DOI":"10.1200\/JCO.21.01728","article-title":"nab-Sirolimus for Patients with Malignant Perivascular Epithelioid Cell Tumors","volume":"39","author":"Wagner","year":"2021","journal-title":"J. Clin. Oncol."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"249","DOI":"10.1586\/17476348.2016.1148603","article-title":"mTOR treatment in lymphangioleiomyomatosis: The role of everolimus","volume":"10","author":"Yates","year":"2016","journal-title":"Expert Rev. Respir. Med."},{"key":"ref_12","doi-asserted-by":"crossref","unstructured":"Cao, B., and Huang, Y. (2022). Malignant perivascular epithelioid cell tumor (PEComa) of the uterus. BMC Women\u2019s Health, 22.","DOI":"10.1186\/s12905-022-02119-9"},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"5295","DOI":"10.1158\/1078-0432.CCR-19-0288","article-title":"Role of Chemotherapy, VEGFR Inhibitors, and mTOR Inhibitors in Advanced Perivascular Epithelioid Cell Tumors (PEComas)","volume":"25","author":"Sanfilippo","year":"2019","journal-title":"Clin. Cancer Res."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"e2200627","DOI":"10.1200\/PO.22.00627","article-title":"Metastatic Malignant Perivascular Epithelioid Cell Tumors with Microsatellite Instability Within Lynch Syndrome Successfully Treated with Anti-PD1 Pembrolizumab","volume":"7","author":"Djerroudi","year":"2023","journal-title":"JCO Precis. Oncol."},{"key":"ref_15","doi-asserted-by":"crossref","unstructured":"Liapi, A., Mathevet, P., Herrera, F.G., Hastir, D., and Sarivalasis, A. (2021). VEGFR Inhibitors for Uterine Metastatic Perivascular Epithelioid Tumors (PEComa) Resistant to mTOR Inhibitors. A Case Report and Review of Literature. Front. Oncol., 11.","DOI":"10.3389\/fonc.2021.641376"},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"9","DOI":"10.1007\/s00292-019-0612-5","article-title":"Complexity of PEComas: Diagnostic approach, molecular background, clinical management","volume":"41","author":"Utpatel","year":"2020","journal-title":"Pathologe"},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"1789","DOI":"10.1007\/s00404-024-07578-z","article-title":"Is there any role for radiotherapy in the management of uterine perivascular epithelioid cell tumors (PEComas)?","volume":"310","author":"Zagardo","year":"2024","journal-title":"Arch. Gynecol. Obstet."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"35","DOI":"10.1016\/j.ygyno.2024.06.007","article-title":"Uterine perivascular epithelioid cell tumors (PEComa) and the accuracy of proposed classification systems in predicting the malignant versus non-malignant behavior","volume":"188","author":"Garzon","year":"2024","journal-title":"Gynecol. Oncol."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"2304","DOI":"10.1002\/cncr.35277","article-title":"PEC-PRO: A new prognostic score from a series of 87 patients with localized perivascular epithelioid cell neoplasms (PEComas) treated with curative intent","volume":"130","author":"Gantzer","year":"2024","journal-title":"Cancer"},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"463","DOI":"10.1007\/s00428-007-0378-7","article-title":"Perivascular epithelioid cell neoplasms (PEComas): Four malignant cases expanding the histopathological spectrum and a description of a unique finding","volume":"450","author":"Weinreb","year":"2007","journal-title":"Virchows Arch."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1155\/2007\/53056","article-title":"PEComa of the Upper Extremity: A Unique Case and Description of an Initial Response to Neoadjuvant Chemotherapy","volume":"2007","author":"Osei","year":"2007","journal-title":"Sarcoma"},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"1622","DOI":"10.1097\/PAS.0b013e3181f5974f","article-title":"PEComa Presenting in Bone: Clinicopathologic Analysis of 6 Cases and Literature Review","volume":"34","author":"Yamashita","year":"2010","journal-title":"Am. J. Surg. Pathol."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"28","DOI":"10.1186\/s13014-018-0974-5","article-title":"Unresectable hepatic PEComa: A rare malignancy treated with stereotactic body radiation therapy (SBRT) followed by complete resection","volume":"13","author":"Kirste","year":"2018","journal-title":"Radiat. Oncol."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"149","DOI":"10.1016\/j.humpath.2017.11.016","article-title":"Malignant perivascular epithelioid cell tumor of the oropharynx with strong TFE3 expression mimicking alveolar soft part sarcoma: A case report and review of the literature","volume":"76","author":"Saluja","year":"2018","journal-title":"Hum. Pathol."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"1088","DOI":"10.1111\/cup.14034","article-title":"Primary cutaneous malignant perivascular epithelioid cell tumor: Case of a rare tumor with review of the literature","volume":"48","author":"Cole","year":"2021","journal-title":"J. Cutan. Pathol."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"2632010x231178629","DOI":"10.1177\/2632010X231178629","article-title":"Primary Cutaneous Malignant Perivascular Epithelio\u00efd Cell Tumor (PEComa): Case Report with Review of the Literature","volume":"16","author":"Elousrouti","year":"2023","journal-title":"Clin. Pathol."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"1036","DOI":"10.1136\/ijgc-00009577-200409000-00045","article-title":"Malignant perivascular epithelioid cell tumor (PEComa) arising in the broad ligament","volume":"14","author":"Fink","year":"2004","journal-title":"Int. J. Gynecol. Cancer"},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"e262","DOI":"10.1016\/j.jaad.2013.06.041","article-title":"Malignant perivascular epithelioid cell tumor: A case report of a cutaneous tumor on the cheek of a male patient","volume":"69","author":"Greveling","year":"2013","journal-title":"J. Am. Acad. Dermatol."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"681","DOI":"10.1097\/01.mph.0000193475.06870.d5","article-title":"Multimodal treatment using surgery, radiotherapy, and chemotherapy in a patient with a perivascular epithelioid cell tumor of the uterus","volume":"27","author":"Jeon","year":"2005","journal-title":"J. Pediatr. Hematol. Oncol."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"e945177","DOI":"10.12659\/AJCR.945177","article-title":"Adrenal PEComa Treated by Surgical Resection and Postoperative Radiotherapy: A Case Report","volume":"25","author":"Akay","year":"2024","journal-title":"Am. J. Case Rep."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"e03200","DOI":"10.1016\/j.heliyon.2020.e03200","article-title":"Malignant perivascular epithelioid cell tumor mimicking jugular foramen schwannoma: A case report and literature review","volume":"6","author":"Komune","year":"2020","journal-title":"Heliyon"},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"e14072","DOI":"10.1097\/MD.0000000000014072","article-title":"Malignant perivascular epithelioid cell tumor in the female genital tract: Preferred reporting items for systematic reviews and meta-analyses","volume":"98","author":"Liu","year":"2019","journal-title":"Medicine"},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"244","DOI":"10.1097\/00000478-200402000-00013","article-title":"Uterine epithelioid leiomyosarcomas with clear cells: Reactivity with HMB-45 and the concept of PEComa","volume":"28","author":"Silva","year":"2004","journal-title":"Am. J. Surg. Pathol."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"1239","DOI":"10.1097\/00000478-200009000-00007","article-title":"Clear cell myomelanocytic tumor of the falciform ligament\/ligamentum teres: A novel member of the perivascular epithelioid clear cell family of tumors with a predilection for children and young adults","volume":"24","author":"Folpe","year":"2000","journal-title":"Am. J. Surg. Pathol."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"341","DOI":"10.1097\/01.pgp.0000168515.83557.89","article-title":"Perivascular epithelioid cell tumor of the uterus: Report of four cases","volume":"24","author":"Fukunaga","year":"2005","journal-title":"Int. J. Gynecol. Pathol."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"493","DOI":"10.1097\/PAS.0b013e318161dc34","article-title":"Sclerosing PEComa: Clinicopathologic analysis of a distinctive variant with a predilection for the retroperitoneum","volume":"32","author":"Hornick","year":"2008","journal-title":"Am. J. Surg. Pathol."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"114","DOI":"10.1159\/000336826","article-title":"Malignant perivascular epithelioid cell tumor of the mesentery: A case report and literature review","volume":"35","author":"Lai","year":"2012","journal-title":"Onkologie"},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"omy115","DOI":"10.1093\/omcr\/omy115","article-title":"Perivascular epithelioid cell tumour and mesonephric adenocarcinoma of the uterine cervix: An unknown co-existence","volume":"2019","author":"Papoutsis","year":"2019","journal-title":"Oxf. Med. Case Rep."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1097\/00000478-200201000-00001","article-title":"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","volume":"26","author":"Vang","year":"2002","journal-title":"Am. J. Surg. Pathol."},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"100657","DOI":"10.1016\/j.adro.2021.100657","article-title":"Molecularly Targeted Radiation Therapy Using mTOR Inhibition for the Management of Malignant Perivascular Epithelioid Cell Tumor (PEComa): A Case Report and Review","volume":"6","author":"Bajaj","year":"2021","journal-title":"Adv. Radiat. Oncol."},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"563","DOI":"10.1038\/modpathol.3880351","article-title":"Abdominopelvic sarcoma of perivascular epithelioid cells. Report of four cases in young women, one with tuberous sclerosis","volume":"14","author":"Bonetti","year":"2001","journal-title":"Mod. Pathol."},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"173","DOI":"10.1016\/j.anndiagpath.2010.02.007","article-title":"Perivascular epithelioid cell tumor of abdominal origin","volume":"14","author":"Bandera","year":"2010","journal-title":"Ann. Diagn. Pathol."},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"54","DOI":"10.1186\/s13000-015-0292-2","article-title":"Malignant perivascular epithelioid cell tumor (PEComa) of the femur: A case report and literature review","volume":"10","author":"Lao","year":"2015","journal-title":"Diagn. Pathol."},{"key":"ref_44","doi-asserted-by":"crossref","unstructured":"Wang, Y., Li, W., Zuo, X., Min, K., Tang, Y., Chen, H., Wang, W., and Zhou, Y. (2023). Anti-PD-1 immunotherapy combined with stereotactic body radiation therapy and GM-CSF for the treatment of advanced malignant PEComa: A case report. Front. Oncol., 13.","DOI":"10.3389\/fonc.2023.1045119"},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"499","DOI":"10.1007\/s00428-017-2208-x","article-title":"Metastatic malignant PEComa of the leg with identification of ATRX mutation by next-generation sequencing","volume":"472","author":"Alnajar","year":"2018","journal-title":"Virchows Arch."},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"383","DOI":"10.1186\/1752-1947-5-383","article-title":"A patient presenting with a perivascular epithelioid cell tumor in the broad ligament: A case report","volume":"5","author":"Ross","year":"2011","journal-title":"J. Med. Case Rep."},{"key":"ref_47","doi-asserted-by":"crossref","unstructured":"Yin, Z., Zhang, H., Zhang, K., Yue, J., Tang, R., Wang, Y., Deng, Q., and Yu, Q. (2025). Impacts of combining PD-L1 inhibitor and radiotherapy on the tumour immune microenvironment in a mouse model of esophageal squamous cell carcinoma. BMC Cancer, 25.","DOI":"10.1186\/s12885-025-13801-0"},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"615","DOI":"10.2147\/CMAR.S351284","article-title":"Management Strategies for Adults with Locally Advanced, Unresectable or Metastatic Malignant Perivascular Epithelioid Cell Tumor (PEComa): Challenges and Solutions","volume":"15","author":"Czarnecka","year":"2023","journal-title":"Cancer Manag. Res."},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"541626","DOI":"10.1155\/2012\/541626","article-title":"\u201cMalignant\u201d perivascular epithelioid cell neoplasm: Risk stratification and treatment strategies","volume":"2012","author":"Bleeker","year":"2012","journal-title":"Sarcoma"}],"container-title":["Cancers"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2072-6694\/18\/9\/1388\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,5,11]],"date-time":"2026-05-11T04:10:35Z","timestamp":1778472635000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2072-6694\/18\/9\/1388"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,4,27]]},"references-count":49,"journal-issue":{"issue":"9","published-online":{"date-parts":[[2026,5]]}},"alternative-id":["cancers18091388"],"URL":"https:\/\/doi.org\/10.3390\/cancers18091388","relation":{},"ISSN":["2072-6694"],"issn-type":[{"value":"2072-6694","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,4,27]]}}}