{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,26]],"date-time":"2026-04-26T07:22:36Z","timestamp":1777188156491,"version":"3.51.4"},"reference-count":18,"publisher":"MDPI AG","issue":"1","license":[{"start":{"date-parts":[[2023,1,11]],"date-time":"2023-01-11T00:00:00Z","timestamp":1673395200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Medicina"],"abstract":"<jats:p>PEComa has become a widely accepted entity, and increased recognition has led to descriptions of this tumor in a wide variety of anatomic sites, including the adrenal gland. PEComa (perivascular epithelioid cell tumor) is a mesenchymal tumor composed of perivascular cells, and the most frequent sites of PEComas are the uterus and retroperitoneum. The incidence is &lt;1 per 1,000,000 people. We report a case of adrenal metastatic PEComa in a 63-year-old man discovered by a spontaneous hematoma of the rectus abdominis. In our case, PEComa of the adrenal gland was a significant diagnostic dilemma as the morphologic and immunophenotypic features of this neoplasm may easily be confused with those of other more commonly encountered lesions.<\/jats:p>","DOI":"10.3390\/medicina59010149","type":"journal-article","created":{"date-parts":[[2023,1,12]],"date-time":"2023-01-12T01:32:26Z","timestamp":1673487146000},"page":"149","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Metastatic Adrenal PEComa: Case Report and Short Review of the Literature"],"prefix":"10.3390","volume":"59","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4781-8013","authenticated-orcid":false,"given":"Enrico","family":"Battistella","sequence":"first","affiliation":[{"name":"Endocrine Surgery Unit, Department of Surgery, Veneto Institute of Oncology, IOV-IRCCS, Via Gattamelata 64, 35128 Padua, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Luca","family":"Pomba","sequence":"additional","affiliation":[{"name":"Endocrine Surgery Unit, Department of Surgery, Veneto Institute of Oncology, IOV-IRCCS, Via Gattamelata 64, 35128 Padua, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7251-8808","authenticated-orcid":false,"given":"Marica","family":"Mirabella","sequence":"additional","affiliation":[{"name":"Endocrine Surgery Unit, Department of Surgery, Veneto Institute of Oncology, IOV-IRCCS, Via Gattamelata 64, 35128 Padua, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michele","family":"Gregianin","sequence":"additional","affiliation":[{"name":"Department of Nuclear Medicine, Veneto Institute of Oncology, IOV-IRCCS, Via Gattamelata 64, 35128 Padua, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Antonio","family":"Scapinello","sequence":"additional","affiliation":[{"name":"Pathology Unit, Veneto Institute of Oncology, IOV-IRCCS, Via Gattamelata 64, 35128 Padua, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marco","family":"Volante","sequence":"additional","affiliation":[{"name":"Department of Oncology, University of Turin, Via Giuseppe Verdi 8, 10124 Turin, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Antonio","family":"Toniato","sequence":"additional","affiliation":[{"name":"Endocrine Surgery Unit, Department of Surgery, Veneto Institute of Oncology, IOV-IRCCS, Via Gattamelata 64, 35128 Padua, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2023,1,11]]},"reference":[{"key":"ref_1","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_2","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_3","first-page":"637","article-title":"A survey on adrenal incidentaloma in Italy","volume":"85","author":"Mantero","year":"2000","journal-title":"J. Clin. Endocrinol. Metab."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"G1","DOI":"10.1530\/EJE-16-0467","article-title":"Management of adrenal incidentalomas: European Society of Endocrinology Clinical Practice Guideline in collaboration with the European Network for the Study of Adrenal Tumors","volume":"175","author":"Fassnacht","year":"2016","journal-title":"Eur. J. Endocrinol."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"200","DOI":"10.3803\/EnM.2017.32.2.200","article-title":"Clinical Guidelines for the Management of Adrenal Incidentaloma","volume":"32","author":"Lee","year":"2017","journal-title":"Endocrinol. Metab."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"503","DOI":"10.1007\/s00464-008-0061-3","article-title":"Factors influencing the rising rates of adrenal surgery: Analysis of a 25-year experience","volume":"23","author":"Toniato","year":"2008","journal-title":"Surg. Endosc."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"775","DOI":"10.1210\/endrev\/bnaa008","article-title":"Adrenal incidentaloma","volume":"41","author":"Sherlock","year":"2020","journal-title":"Endocr. 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Med."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"113","DOI":"10.1016\/j.prp.2011.11.002","article-title":"Malignant PEComa of the adrenal gland","volume":"208","author":"Lau","year":"2012","journal-title":"Pathol. Res. Pract."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/j.ijsu.2015.05.002","article-title":"Perivascular epithelioid cell neoplasm (PEComa) of the uterus: A systematic review","volume":"19","author":"Musella","year":"2015","journal-title":"Int. J. Surg."},{"key":"ref_13","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_14","doi-asserted-by":"crossref","first-page":"7","DOI":"10.1186\/1477-7800-5-7","article-title":"Uterine PEComa: Appraisal of a controversial and increasingly reported mesenchymal neoplasm","volume":"5","author":"Fadare","year":"2008","journal-title":"Int. Semin. Surg. Oncol."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"677","DOI":"10.1016\/S0090-8258(03)00325-1","article-title":"Recurrent perivascular epithelioid cell tumor of the uterus (PEComa): An immunohistochemical study and review of the literature","volume":"90","author":"Greene","year":"2003","journal-title":"Gynecol. Oncol."},{"key":"ref_16","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."},{"key":"ref_17","first-page":"5204092","article-title":"Angiomyolipoma and Malignant PEComa: Discussion of Two Rare Adrenal Tumors","volume":"2016","author":"Ii","year":"2016","journal-title":"Case Rep. Oncol. Med."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"502","DOI":"10.1007\/s12020-013-0082-0","article-title":"A very rare bilateral adrenal tumor","volume":"45","author":"Toniato","year":"2013","journal-title":"Endocrine"}],"container-title":["Medicina"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/1648-9144\/59\/1\/149\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T18:03:39Z","timestamp":1760119419000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/1648-9144\/59\/1\/149"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,1,11]]},"references-count":18,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2023,1]]}},"alternative-id":["medicina59010149"],"URL":"https:\/\/doi.org\/10.3390\/medicina59010149","relation":{},"ISSN":["1648-9144"],"issn-type":[{"value":"1648-9144","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,1,11]]}}}