{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,22]],"date-time":"2026-10-22T08:40:39Z","timestamp":1792658439888,"version":"4.5.0"},"reference-count":37,"publisher":"Oxford University Press (OUP)","issue":"1187","license":[{"start":{"date-parts":[[2026,7,2]],"date-time":"2026-07-02T00:00:00Z","timestamp":1782950400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"name":"Canadian Sarcoma Research and Clinical Collaboration"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2026,11,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Objectives<\/jats:title>\n                    <jats:p>Perivascular epithelioid cell tumors (PEComas) are ultra-rare soft tissue tumors with heterogeneous treatment approaches. While surgery is the standard for localized disease, the role of radiotherapy (RT) remains unclear.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>This retrospective study aims to evaluate tumor volume response, as well as local outcomes and OS, in patients with PEComa treated with RT. The cohort included patients diagnosed with PEComa between 2005 and 2023 at a single sarcoma center. Demographic, clinical, RT, and volumetric tumor data were collected. Response was assessed using RECIST v1.1.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>In total, 24 lesions in 12 patients were evaluable for analysis, with a median radiological follow-up of 10\u2009months. Of these, 7 lesions were treated with perioperative RT and 17 with palliative RT. Systemic treatments during imaging follow-up were administered in 19\/24 lesions. Among 17 lesions treated with palliative intent without surgery, 4 local failures occurred\u2014all at EQD2 (\u03b1\/\u03b2\u2009=\u20095) &amp;lt;40\u2009Gy\u2014and the estimated 1-year local control rate was 61.9%. Among these 17 lesions, mean tumor volume decreased from 301.5\u2009cc (range: 0.9-2251) to 108.3\u2009cc (range: 0.1-754) as best response after RT. Seven patients (58.3%) died from their disease, with a median OS of 46\u2009months after diagnosis.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>PEComas appear to be radiosensitive, with substantial tumor volume reduction after RT and a dose-response signal favoring higher EQD2 dose. Nonetheless, OS was poor, reflecting the aggressive nature of this disease.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Advances in knowledge<\/jats:title>\n                    <jats:p>This is the largest RT series in PEComa to date and suggests radiosensitivity in this disease.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1093\/bjr\/tqag159","type":"journal-article","created":{"date-parts":[[2026,7,2]],"date-time":"2026-07-02T15:33:39Z","timestamp":1783006419000},"page":"2308-2315","source":"Crossref","is-referenced-by-count":0,"title":["An evaluation of radiotherapy and response in the management of perivascular epithelioid cell tumors"],"prefix":"10.1093","volume":"99","author":[{"ORCID":"https:\/\/orcid.org\/0009-0009-9686-5802","authenticated-orcid":false,"given":"Reinhardt","family":"Krcek","sequence":"first","affiliation":[{"name":"Radiation Medicine Program, Princess Margaret Cancer Centre, University Health Network , Toronto, ON, M5G 2M9,","place":["Canada"]},{"name":"Department of Radiation Oncology, University Hospital Zurich, University of Zurich , Zurich, 8091,","place":["Switzerland"]},{"name":"University of Toronto , Toronto, ON, M5S 1A1,","place":["Canada"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kristina","family":"Nesterova","sequence":"additional","affiliation":[{"name":"University of Toronto , Toronto, ON, M5S 1A1,","place":["Canada"]},{"name":"Department of Medical Oncology and Hematology, Princess Margaret Cancer Centre, University Health Network , Toronto, ON, M5G 2M9,","place":["Canada"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anna Theresa","family":"Santiago","sequence":"additional","affiliation":[{"name":"Department of Biostatistics, Princess Margaret Cancer Centre, University Health Network , Toronto, ON, M5G 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20251,","place":["Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hagit","family":"Peretz","sequence":"additional","affiliation":[{"name":"Department of Medical Oncology and Hematology, Princess Margaret Cancer Centre, University Health Network , Toronto, ON, M5G 2M9,","place":["Canada"]},{"name":"Division of Hematology\/Oncology, Hospital for Sick Children , Toronto, ON, M5G 1E8,","place":["Canada"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Abha","family":"Gupta","sequence":"additional","affiliation":[{"name":"University of Toronto , Toronto, ON, M5S 1A1,","place":["Canada"]},{"name":"Department of Medical Oncology and Hematology, Princess Margaret Cancer Centre, University Health Network , Toronto, ON, M5G 2M9,","place":["Canada"]},{"name":"Division of Hematology\/Oncology, Hospital for Sick Children , Toronto, ON, M5G 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