{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,7,30]],"date-time":"2025-07-30T12:14:22Z","timestamp":1753877662120,"version":"3.41.2"},"reference-count":6,"publisher":"Oxford University Press (OUP)","issue":"3","license":[{"start":{"date-parts":[[2020,3,30]],"date-time":"2020-03-30T00:00:00Z","timestamp":1585526400000},"content-version":"vor","delay-in-days":29,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2020,3,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>Intra-abdominal perivascular epithelioid cell tumors (PEComas) are rare mesenchymal tumors. Although no effective therapies have been agreed upon, mTOR inhibitors are currently being investigated as a potential therapy for this extremely rare tumor. We present a case of a 64-year-old male found to have a large intra-abdominal PEComa with multiple metastatic lesions in the liver. Patient underwent surgical resection of the primary lesion in the abdomen and sigmoid colon followed by adjuvant therapy with the mTOR inhibitor, sirolimus. Initial response was noted with a decrease in size and number of lesions found in the patient\u2019s liver. After 8\u00a0months of therapy, restaging imaging showed disease progression in the liver lesions. Patient subsequently failed treatments with pazopanib, investigational therapy TAK-228 (Sapanisertib) and nivolumab and ipilimumab. Overall the patient died after 22\u00a0months of disease. PEComas generally follow a benign course. This case is a much rarer entity given the malignant features\/outcome.<\/jats:p>","DOI":"10.1093\/omcr\/omaa013","type":"journal-article","created":{"date-parts":[[2020,3,30]],"date-time":"2020-03-30T09:25:08Z","timestamp":1585560308000},"source":"Crossref","is-referenced-by-count":7,"title":["Rapidly enlarging malignant abdominal PEComa with hepatic metastasis: a promising initial response to sirolimus following surgical excision of primary tumor"],"prefix":"10.1093","volume":"2020","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1509-2203","authenticated-orcid":false,"given":"Dustin J","family":"Uhlenhopp","sequence":"first","affiliation":[{"name":"Department of Internal Medicine, MercyOne Des Moines Medical Center, Des Moines, IA 50314, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jacob","family":"West","sequence":"first","affiliation":[{"name":"Department of Internal Medicine, MercyOne Des Moines Medical Center, Des Moines, IA 50314, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jonathan","family":"Heckart","sequence":"first","affiliation":[{"name":"Department of Internal Medicine, MercyOne Des Moines Medical Center, Des Moines, IA 50314, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ruth","family":"Campbell","sequence":"first","affiliation":[{"name":"Department of Internal Medicine, MercyOne Des Moines Medical Center, Des Moines, IA 50314, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Abdelaziz","family":"Elhaddad","sequence":"first","affiliation":[{"name":"Department of Hematology\/Oncology, MercyOne Cancer Center, Des Moines, IA 50314, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[2020,3,30]]},"reference":[{"key":"2020033005250287100_ref1","doi-asserted-by":"publisher","first-page":"113","DOI":"10.1186\/s12957-018-1407-5","article-title":"A rare case of perivascular epithelioid cell tumor (PEComa) of the greater omentum","volume":"16","author":"Okamoto","year":"2018","journal-title":"World J Surg Oncol"},{"key":"2020033005250287100_ref2","first-page":"2251","article-title":"Malignant perivascular epithelioid cell tumor of the retroperitoneum","volume":"6","author":"Wu","year":"2013","journal-title":"Int J Clin Exp Pathol"},{"key":"2020033005250287100_ref3","doi-asserted-by":"crossref","first-page":"835","DOI":"10.1200\/JCO.2009.25.2981","article-title":"Clinical activity of mTOR inhibition with sirolimus in malignant perivascular epithelioid cell tumors: targeting the pathogenic activation of mTORC1 in tumors","volume":"28","author":"Wagner","year":"2010","journal-title":"J Clin Oncol"},{"key":"2020033005250287100_ref4","doi-asserted-by":"publisher","first-page":"541626","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":"2020033005250287100_ref5","doi-asserted-by":"publisher","first-page":"1657","DOI":"10.3748\/wjg.v19.i10.1657","article-title":"PEComa of the colon resistant to sirolimus but responsive to doxorubicin\/ifosfamide","volume":"19","author":"Scheppach","year":"2013","journal-title":"World J Gastroenterol"},{"key":"2020033005250287100_ref6","doi-asserted-by":"crossref","first-page":"6161","DOI":"10.21873\/anticanres.11208","article-title":"Treatment of advanced malignant uterine perivascular Epithelioid cell tumor with mTOR inhibitors: single-institution experience and review of the literature","volume":"36","author":"Starbuck","year":"2016","journal-title":"Anticancer Res"}],"container-title":["Oxford Medical Case Reports"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/academic.oup.com\/omcr\/article-pdf\/2020\/3\/omaa013\/32979507\/omaa013.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"http:\/\/academic.oup.com\/omcr\/article-pdf\/2020\/3\/omaa013\/32979507\/omaa013.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2020,3,30]],"date-time":"2020-03-30T09:25:10Z","timestamp":1585560310000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/omcr\/article\/doi\/10.1093\/omcr\/omaa013\/5813551"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,3,1]]},"references-count":6,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2020,3,1]]}},"URL":"https:\/\/doi.org\/10.1093\/omcr\/omaa013","relation":{},"ISSN":["2053-8855"],"issn-type":[{"type":"electronic","value":"2053-8855"}],"subject":[],"published-other":{"date-parts":[[2020,3]]},"published":{"date-parts":[[2020,3,1]]},"article-number":"omaa013"}}