{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,3]],"date-time":"2026-10-03T17:36:14Z","timestamp":1791048974703,"version":"4.1.0"},"reference-count":51,"publisher":"Elsevier BV","issue":"3","license":[{"start":{"date-parts":[[2018,4,1]],"date-time":"2018-04-01T00:00:00Z","timestamp":1522540800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2018,4,1]],"date-time":"2018-04-01T00:00:00Z","timestamp":1522540800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"}],"content-domain":{"domain":["clinicalkey.com","clinicalkey.com.au","clinicalkey.es","clinicalkey.fr","clinicalkey.jp","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Pancreatology"],"published-print":{"date-parts":[[2018,4]]},"DOI":"10.1016\/j.pan.2018.02.007","type":"journal-article","created":{"date-parts":[[2018,2,19]],"date-time":"2018-02-19T02:38:37Z","timestamp":1519007917000},"page":"238-245","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":19,"title":["Primary pancreatic perivascular epithelioid cell tumor (PEComa): A surgical enigma. A systematic review of the literature"],"prefix":"10.1016","volume":"18","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9841-7856","authenticated-orcid":false,"given":"Maurizio","family":"Zizzo","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lara","family":"Ugoletti","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David","family":"Tumiati","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carolina","family":"Castro Ruiz","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stefano","family":"Bonacini","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michele","family":"Panebianco","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giuliana","family":"Sereni","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Antonio","family":"Manenti","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Filippo","family":"Lococo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gabriele","family":"Carlinfante","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Claudio","family":"Pedrazzoli","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.pan.2018.02.007_bib1","doi-asserted-by":"crossref","first-page":"359","DOI":"10.1016\/j.anndiagpath.2015.06.003","article-title":"PEComa: morphology and genetics of a complex tumor family","volume":"19","author":"Thway","year":"2015","journal-title":"Ann Diagn Pathol"},{"key":"10.1016\/j.pan.2018.02.007_bib2","series-title":"Pathology and genetics of tumours of soft tissue and bone","article-title":"World Health Organization classification of tumours","year":"2002"},{"key":"10.1016\/j.pan.2018.02.007_bib3","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":"10.1016\/j.pan.2018.02.007_bib4","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":"10.1016\/j.pan.2018.02.007_bib5","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/j.humpath.2009.05.011","article-title":"Perivascular epithelioid cell neoplasms: pathology and pathogenesis","volume":"41","author":"Folpe","year":"2010","journal-title":"Hum Pathol"},{"key":"10.1016\/j.pan.2018.02.007_bib6","doi-asserted-by":"crossref","first-page":"119","DOI":"10.1007\/s00428-007-0509-1","article-title":"PEComas: the past, the present and the future","volume":"452","author":"Martignoni","year":"2008","journal-title":"Virchows Arch"},{"key":"10.1016\/j.pan.2018.02.007_bib7","first-page":"28","article-title":"A\u00a0systematic review: perivascular epithelioid cell tumor of gastrointestinal tract","volume":"95","author":"Chen","year":"2016","journal-title":"Medicine"},{"key":"10.1016\/j.pan.2018.02.007_bib8","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":"10.1016\/j.pan.2018.02.007_bib9","doi-asserted-by":"crossref","first-page":"443","DOI":"10.1097\/00006676-200405000-00014","article-title":"Primary angiomyolipoma of the pancreas","volume":"28","author":"Heywood","year":"2004","journal-title":"Pancreas"},{"key":"10.1016\/j.pan.2018.02.007_bib10","doi-asserted-by":"crossref","first-page":"555","DOI":"10.1007\/s00428-005-1216-4","article-title":"\u201cSugar\u201d tumor of the pancreas: a rare entity that is diagnosable on preoperative fine-needle biopsies","volume":"446","author":"Ramuz","year":"2005","journal-title":"Virchows Arch"},{"key":"10.1016\/j.pan.2018.02.007_bib11","first-page":"138","article-title":"Pancreatic perivascular epithelioid cell tumor (PEComa)","volume":"28","author":"Perigny","year":"2008","journal-title":"Ann Pathol"},{"key":"10.1016\/j.pan.2018.02.007_bib12","doi-asserted-by":"crossref","first-page":"650","DOI":"10.1111\/j.1440-1827.2009.02421.x","article-title":"Perivascular epithelioid cell tumor (PEComa) of the pancreas: immunoelectron microscopy and review of the literature","volume":"59","author":"Hirabayashi","year":"2009","journal-title":"Pathol Int"},{"key":"10.1016\/j.pan.2018.02.007_bib13","first-page":"679","article-title":"Pancreatic PEComa (sugar tumor): MDCT and EUS features","volume":"10","author":"Baez","year":"2009","journal-title":"JOP"},{"key":"10.1016\/j.pan.2018.02.007_bib14","first-page":"55","article-title":"Asymptomatic pancreatic perivascular epithelial cell tumor (PEComa) in a male patient: report and literature review","volume":"12","author":"Zemet","year":"2011","journal-title":"JOP"},{"key":"10.1016\/j.pan.2018.02.007_bib15","doi-asserted-by":"crossref","first-page":"1155","DOI":"10.1097\/MPA.0b013e318221fc0e","article-title":"Perivascular epithelioid cell neoplasm (PEComa) originating from the pancreas and metastasizing to the liver","volume":"40","author":"Nagata","year":"2011","journal-title":"Pancreas"},{"issue":"9","key":"10.1016\/j.pan.2018.02.007_bib16","first-page":"964","article-title":"Clinicopathologic features of perivascular epithelioid cell tumor of the pancreas","volume":"19","author":"Xie","year":"2011","journal-title":"Shijie Huaren Xiaohua Zazhi"},{"key":"10.1016\/j.pan.2018.02.007_bib17","doi-asserted-by":"crossref","first-page":"124","DOI":"10.3109\/01913123.2011.642463","article-title":"Pancreatic PEComa: a case report with ultrastructural localization of HMB-45 within melanosomes","volume":"36","author":"Finzi","year":"2012","journal-title":"Ultrastruct Pathol"},{"key":"10.1016\/j.pan.2018.02.007_bib18","doi-asserted-by":"crossref","first-page":"336","DOI":"10.1007\/s12029-010-9183-7","article-title":"Solitary lymphangioleiomyoma of pancreas mimicking pancreatic pseudocyst-a case report","volume":"43","author":"Singh","year":"2012","journal-title":"J\u00a0Gastrointest Canc"},{"issue":"1","key":"10.1016\/j.pan.2018.02.007_bib19","doi-asserted-by":"crossref","first-page":"165","DOI":"10.1016\/j.gie.2013.02.011","article-title":"Perivascular epithelioid cell tumor: an unusual pancreatic mass diagnosed by EUS-FNA","volume":"78","author":"Al-Haddad","year":"2013","journal-title":"Gastrointest Endosc"},{"key":"10.1016\/j.pan.2018.02.007_bib20","first-page":"2061","article-title":"A\u00a0resected perivascular epithelioid cell tumor (PEComa) of the pancreas diagnosed using endoscopic ultrasound-guided fine-needle aspiration","volume":"52","author":"Okuwaki","year":"2013","journal-title":"Integr Med"},{"key":"10.1016\/j.pan.2018.02.007_bib21","doi-asserted-by":"crossref","first-page":"e1","DOI":"10.1097\/PAI.0b013e3182392bb6","article-title":"Perivascular epithelioid cell tumor: the first malignant case report in the pancreas","volume":"21","author":"Mourra","year":"2013","journal-title":"Appl Immunohistochem Mol Morphol"},{"key":"10.1016\/j.pan.2018.02.007_bib22","doi-asserted-by":"crossref","DOI":"10.1038\/ctg.2013.15","article-title":"Differential diagnosis of focal non-cystic pancreatic lesions with and without proximal dilation of pancreatic duct noted on CT scan","volume":"4","author":"Tummala","year":"2013","journal-title":"Clin Transl Gastroenterol"},{"key":"10.1016\/j.pan.2018.02.007_bib23","doi-asserted-by":"crossref","first-page":"959","DOI":"10.1097\/MPA.0000000000000130","article-title":"Primary mesenchymal tumors of the pancreas: single-center experience over 16 years","volume":"43","author":"Kim","year":"2014","journal-title":"Pancreas"},{"key":"10.1016\/j.pan.2018.02.007_bib24","article-title":"Pancreatic perivascular epithelioid cell tumour presenting with upper gastrointestinal bleeding","author":"Petrides","year":"2015","journal-title":"Case Rep Oncol Med"},{"key":"10.1016\/j.pan.2018.02.007_bib25","article-title":"Primary perivascular epithelioid cell tumor of the pancreas: a case report","volume":"S30","author":"Wei","year":"2016","journal-title":"Pancreatology"},{"key":"10.1016\/j.pan.2018.02.007_bib26","doi-asserted-by":"crossref","first-page":"3693","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":"10.1016\/j.pan.2018.02.007_bib27","doi-asserted-by":"crossref","first-page":"59","DOI":"10.1186\/s40792-016-0186-x","article-title":"Perivascular epithelial cell tumor (PEComa) of the pancreas: a case report and review of previous literatures","volume":"2","author":"Mizuuchi","year":"2016","journal-title":"Surg Case Rep"},{"key":"10.1016\/j.pan.2018.02.007_bib28","doi-asserted-by":"crossref","first-page":"59","DOI":"10.1002\/dc.23599","article-title":"Perivascular epithelioid cell tumor (PEComa) of pancreas diagnosed preoperatively by endoscopic ultrasound-guided fine-needle aspiration: a case report and review of literature","volume":"45","author":"Collins","year":"2017","journal-title":"Diagn Cytopathol"},{"key":"10.1016\/j.pan.2018.02.007_bib29","doi-asserted-by":"crossref","first-page":"707","DOI":"10.1007\/s00428-016-2032-8","article-title":"Pancreatic PEComa is a novel member of the family of tuberous sclerosis complex-associated tumors: case report and review of the literature","volume":"469","author":"Hartley","year":"2016","journal-title":"Virchows Arch"},{"issue":"1","key":"10.1016\/j.pan.2018.02.007_bib30","first-page":"92","article-title":"Primary pancreatic PEComa: is surgery always the best choice?","volume":"18","author":"Zizzo","year":"2017","journal-title":"JOP"},{"key":"10.1016\/j.pan.2018.02.007_bib31","first-page":"22","article-title":"Perivascular epithelial cell tumor (PEComa) of the pancreas: a case report and review of literature","volume":"96","author":"Zhang","year":"2017","journal-title":"Medicine"},{"issue":"7","key":"10.1016\/j.pan.2018.02.007_bib32","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pmed.1000097","article-title":"Preferred reporting items for systematic reviews and meta-analyses: the PRISMA statement","volume":"6","author":"Moher","year":"2009","journal-title":"PLoS Med"},{"key":"10.1016\/j.pan.2018.02.007_bib33","doi-asserted-by":"crossref","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":"10.1016\/j.pan.2018.02.007_bib34","doi-asserted-by":"crossref","first-page":"648","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":"10.1016\/j.pan.2018.02.007_bib35","doi-asserted-by":"crossref","first-page":"751","DOI":"10.5858\/2001-125-0751-RA","article-title":"Renal angiomyolipoma: further immunophenotypic characterization of an expanding morphologic spectrum","volume":"125","author":"Stone","year":"2001","journal-title":"Arch Pathol Lab Med"},{"key":"10.1016\/j.pan.2018.02.007_bib36","first-page":"7","article-title":"Evidence on the neural crest origin of PEComas","volume":"52","author":"Fernandez-Flores","year":"2011","journal-title":"Rom J Morphol Embryol"},{"key":"10.1016\/j.pan.2018.02.007_bib37","doi-asserted-by":"crossref","first-page":"2569","DOI":"10.1111\/j.1582-4934.2011.01461.x","article-title":"Telocytes are the common cell of origin of both PEComas and GISTs: an evidence-supported hypothesis","volume":"15","author":"Ardeleanu","year":"2011","journal-title":"J\u00a0Cell Mol Med"},{"key":"10.1016\/j.pan.2018.02.007_bib38","doi-asserted-by":"crossref","first-page":"21","DOI":"10.1080\/019131201300004654","article-title":"Angiomyolipoma: immunohistochemical and ultrastructural study of 14 cases","volume":"25","author":"Barnard","year":"2001","journal-title":"Ultrastruct Pathol"},{"issue":"10","key":"10.1016\/j.pan.2018.02.007_bib39","first-page":"13023","article-title":"Expression of CD44 and CD29 by PEComa cells suggests their possible origin of mesenchymal stem cells","volume":"8","author":"Liu","year":"2015","journal-title":"Int J Clin Exp Pathol"},{"key":"10.1016\/j.pan.2018.02.007_bib40","doi-asserted-by":"crossref","first-page":"555","DOI":"10.1016\/j.crad.2012.10.021","article-title":"Perivascular epithelioid cell tumour: dynamic CT, MRI and clinicopathological characteristicsdAnalysis of 32 cases and review of the literature","volume":"68","author":"Tan","year":"2013","journal-title":"Clin Radiol"},{"key":"10.1016\/j.pan.2018.02.007_bib41","doi-asserted-by":"crossref","first-page":"368","DOI":"10.1007\/s00261-015-0568-8","article-title":"Update on the imaging of malignant perivascular epithelioid cell tumors (PEComas)","volume":"41","author":"Phillips","year":"2016","journal-title":"Abdom Radiol"},{"key":"10.1016\/j.pan.2018.02.007_bib42","doi-asserted-by":"crossref","first-page":"123","DOI":"10.1148\/rg.2016150212","article-title":"Nonepithelial Neoplasms of the pancreas: radiologic-pathologic correlation, part 1-Benign tumors","volume":"36","author":"Manning","year":"2016","journal-title":"Radiographics"},{"key":"10.1016\/j.pan.2018.02.007_bib43","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":"10.1016\/j.pan.2018.02.007_bib44","doi-asserted-by":"crossref","DOI":"10.1155\/2012\/541626","article-title":"\u201cMalignant\u201d perivascular epithelioid cell neoplasm: risk stratification and treatment strategies","author":"Bleeker","year":"2012","journal-title":"Sarcoma"},{"key":"10.1016\/j.pan.2018.02.007_bib45","doi-asserted-by":"crossref","first-page":"1361","DOI":"10.1016\/S0002-9440(10)62563-1","article-title":"Solidpseudopapillary tumors of the pancreas are genetically distinct from pancreatic ductal adenocarcinomas and almost always harbor beta-catenin mutations","volume":"160","author":"Abraham","year":"2002","journal-title":"Am J Pathol"},{"key":"10.1016\/j.pan.2018.02.007_bib46","doi-asserted-by":"crossref","first-page":"194","DOI":"10.1097\/01.pas.0000184819.71752.ad","article-title":"Lipid-rich variant of pancreatic endocrine neoplasms","volume":"30","author":"Singh","year":"2006","journal-title":"Am J Surg Pathol"},{"key":"10.1016\/j.pan.2018.02.007_bib47","doi-asserted-by":"crossref","first-page":"1049","DOI":"10.1016\/j.hoc.2013.07.009","article-title":"Targeted therapies in rare sarcomas: IMT, ASPS, SFT, PEComa, and CCS","volume":"27","author":"Stacchiotti","year":"2013","journal-title":"Hematol Oncol Clin N Am"},{"key":"10.1016\/j.pan.2018.02.007_bib48","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\u00a0Clin Oncol"},{"key":"10.1016\/j.pan.2018.02.007_bib49","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"},{"key":"10.1016\/j.pan.2018.02.007_bib50","doi-asserted-by":"crossref","first-page":"814","DOI":"10.1093\/jjco\/hyr035","article-title":"Dramatic tumor response to everolimus for malignant epithelioid angiomyolipoma","volume":"41","author":"Shitara","year":"2011","journal-title":"Jpn J Clin Oncol"},{"key":"10.1016\/j.pan.2018.02.007_bib51","doi-asserted-by":"crossref","DOI":"10.1200\/JCO.2010.29.4678","article-title":"Resistance to mammalian target of rapamycin inhibitor therapy in perivascular epithelioid cell tumors","volume":"28","author":"Subbiah","year":"2010","journal-title":"J\u00a0Clin Oncol"}],"container-title":["Pancreatology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1424390318300310?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1424390318300310?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,10,25]],"date-time":"2025-10-25T16:06:26Z","timestamp":1761408386000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S1424390318300310"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018,4]]},"references-count":51,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2018,4]]}},"alternative-id":["S1424390318300310"],"URL":"https:\/\/doi.org\/10.1016\/j.pan.2018.02.007","relation":{},"ISSN":["1424-3903"],"issn-type":[{"value":"1424-3903","type":"print"}],"subject":[],"published":{"date-parts":[[2018,4]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Primary pancreatic perivascular epithelioid cell tumor (PEComa): A surgical enigma. A systematic review of the literature","name":"articletitle","label":"Article Title"},{"value":"Pancreatology","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.pan.2018.02.007","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2018 IAP and EPC. Published by Elsevier B.V. All rights reserved.","name":"copyright","label":"Copyright"}]}}