{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,20]],"date-time":"2026-05-20T05:08:01Z","timestamp":1779253681085,"version":"3.51.4"},"reference-count":11,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2026,5,20]],"date-time":"2026-05-20T00:00:00Z","timestamp":1779235200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Oncol."],"abstract":"<jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Perivascular epithelioid cell tumors (PEComas) are rare mesenchymal neoplasms with variable malignant potential, and gastrointestinal involvement is particularly uncommon. Colonic PEComas are exceedingly rare and may present with nonspecific symptoms, complicating early diagnosis.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Case presentation<\/jats:title>\n                    <jats:p>A 58-year-old male presented with prolonged fever, fatigue, and weight loss. Laboratory findings revealed leukocytosis and anemia, while infectious and rheumatologic evaluations were unremarkable. Following rectal bleeding, colonoscopy identified a perforated lesion in the sigmoid colon. Imaging demonstrated extensive tumoral wall thickening, and the patient underwent emergency surgery due to acute abdomen. Histopathological and immunohistochemical analysis confirmed malignant PEComa. The patient received adjuvant chemotherapy and radiotherapy and remained disease-free at 5-year follow-up.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>This case highlights an unusual presentation of colonic PEComa with fever of unknown origin and spontaneous perforation. Clinicians should consider rare malignancies in patients with unexplained inflammatory syndromes, particularly when accompanied by gastrointestinal findings.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.3389\/fonc.2026.1852776","type":"journal-article","created":{"date-parts":[[2026,5,20]],"date-time":"2026-05-20T04:31:41Z","timestamp":1779251501000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":0,"title":["Case Report: Malignant colonic PEComa presenting as fever of unknown origin with spontaneous perforation"],"prefix":"10.3389","volume":"16","author":[{"given":"Gizem","family":"Gunes","sequence":"first","affiliation":[{"name":"Department of Surgical Oncology, Ankara Bilkent City Hospital","place":["Ankara, T\u00fcrkiye"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Adem","family":"Ozcan","sequence":"additional","affiliation":[{"name":"Department of Surgical Oncology, Ankara Bilkent City Hospital","place":["Ankara, T\u00fcrkiye"]}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1965","published-online":{"date-parts":[[2026,5,20]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0b013e31829caab3","article-title":"Pecoma of the gastrointestinal tract: clinicopathologic study of 35 cases with evaluation of prognostic parameters","volume":"37","author":"Doyle","year":"2013","journal-title":"Am J Surg Pathol"},{"key":"B2","doi-asserted-by":"publisher","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":"B3","doi-asserted-by":"publisher","DOI":"10.1155\/2012\/541626","article-title":"Malignant perivascular epithelioid cell neoplasm: risk stratification and treatment strategies","volume":"2012","author":"Bleeker","year":"2012","journal-title":"Sarcoma"},{"key":"B4","doi-asserted-by":"publisher","DOI":"10.1177\/03000605211041509","article-title":"Unusual paediatric sigmoid perivascular epithelioid cell tumour with regional lymph node metastasis treated using gemcitabine and docetaxel: a case report and literature review","volume":"49","author":"Cheng","year":"2021","journal-title":"J Int Med Res"},{"key":"B5","doi-asserted-by":"publisher","DOI":"10.3748\/wjg.v22.i13.3693","article-title":"Pancreatic perivascular epithelioid cell tumor: a case report with clinicopathological features and a literature review","volume":"22","author":"Jiang","year":"2016","journal-title":"World J Gastroenterol"},{"key":"B6","doi-asserted-by":"publisher","DOI":"10.5858\/133.4.648","article-title":"Perivascular epithelioid cell tumor","volume":"133","author":"Armah","year":"2009","journal-title":"Arch Pathol Lab Med"},{"key":"B7","doi-asserted-by":"publisher","first-page":"75","DOI":"10.1111\/j.1365-2559.2005.02316.x","article-title":"Pecoma: what do we know so far","volume":"48","author":"Hornick","year":"2006","journal-title":"Histopathology"},{"key":"B8","doi-asserted-by":"publisher","DOI":"10.3390\/curroncol32060330","article-title":"Perivascular epithelioid cell tumor (PEComa) of the sigmoid colon: case report and literature review","volume":"32","author":"Slice","year":"2025","journal-title":"Curr Oncol"},{"key":"B9","doi-asserted-by":"publisher","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":"B10","doi-asserted-by":"publisher","DOI":"10.3389\/fonc.2021.641376","article-title":"Vegfr inhibitors for uterine metastatic perivascular epithelioid tumors (PEComa) resistant to mTOR inhibitors: a case report and review of the literature","volume":"11","author":"Liapi","year":"2021","journal-title":"Front Oncol"},{"key":"B11","doi-asserted-by":"publisher","DOI":"10.4251\/wjgo.v17.i2.99662","article-title":"Malignant perivascular epithelioid cell tumor of the colorectum: clinicopathological characterization, diagnosis and treatment process of 7 cases","volume":"17","author":"Ma","year":"2025","journal-title":"World J Gastrointest Oncol"}],"container-title":["Frontiers in Oncology"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fonc.2026.1852776\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,5,20]],"date-time":"2026-05-20T04:32:08Z","timestamp":1779251528000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fonc.2026.1852776\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,5,20]]},"references-count":11,"alternative-id":["10.3389\/fonc.2026.1852776"],"URL":"https:\/\/doi.org\/10.3389\/fonc.2026.1852776","relation":{},"ISSN":["2234-943X"],"issn-type":[{"value":"2234-943X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,5,20]]},"article-number":"1852776"}}