{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,19]],"date-time":"2026-10-19T15:23:24Z","timestamp":1792423404021,"version":"4.5.0"},"reference-count":16,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2022,12,15]],"date-time":"2022-12-15T00:00:00Z","timestamp":1671062400000},"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. Med."],"abstract":"<jats:p>Pulmonary epithelioid hemangioendothelioma (PEH) is a rare vascular tumor of endothelial origin with low- to intermediate-grade malignant potentials. Since there is no characteristic clinical or biological marker available for PEH, most cases require a surgical lung biopsy for diagnosis. To date, although some patients with PEH reported in the literature were diagnosed through bronchoscopic biopsy, most of the patients still underwent surgical lung biopsy for confirmation. In this case report, we present a rare case diagnosed as PEH through endobronchial biopsies due to the presence of an intraluminal mass that blocked the trachea and caused atelectasis in the right upper lobe. Moreover, since surgery was not appropriate for this patient with unresectable bilateral multiple nodules, we adopted genetic analysis using NGS to provide a guide for personalized treatment. Then, based on the NGS results, the patient was treated with anti-PD-1 mAb and sirolimus for 1 year and has been stable in a 1-year follow-up examination.<\/jats:p>","DOI":"10.3389\/fmed.2022.1066870","type":"journal-article","created":{"date-parts":[[2022,12,15]],"date-time":"2022-12-15T06:48:59Z","timestamp":1671086939000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":5,"title":["Case report: Rare epithelioid hemangioendothelioma occurs in both main bronchus and lung"],"prefix":"10.3389","volume":"9","author":[{"given":"Jiuyu","family":"Gong","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fangfang","family":"Tian","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Qin","family":"Wang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mi","family":"Mu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sijia","family":"Geng","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pengfei","family":"Hao","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pengfei","family":"Zhong","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rui","family":"Zhang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lin","family":"Jiang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rentao","family":"Wang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pengtao","family":"Bao","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2022,12,15]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","first-page":"89","DOI":"10.1183\/09031936.98.12010089","article-title":"Pulmonary epithelioid haemangioendothelioma in 21 patients, including three with partial spontaneous regression.","volume":"12","author":"Kitaichi","year":"1998","journal-title":"Eur Respir J."},{"key":"B2","first-page":"221","article-title":"Pulmonary epithelioid haemangioendothelioma studies in vitro and in vivo: new diagnostic and treatment methods.","volume":"27","author":"P\u00e1Lf\u00f6ldi","year":"2013","journal-title":"In Vivo."},{"key":"B3","doi-asserted-by":"crossref","first-page":"788","DOI":"10.1097\/RCT.0000000000001194","article-title":"Pulmonary epithelioid hemangioendothelioma: imaging and clinical features.","volume":"45","author":"Liu","year":"2021","journal-title":"J Compu Assist Tomogr."},{"key":"B4","doi-asserted-by":"publisher","first-page":"300060520913148","DOI":"10.1177\/0300060520913148","article-title":"Multiple bilateral pulmonary epithelioid hemangioendothelioma mimicking metastatic lung cancer: case report and literature review.","volume":"48","author":"Xiong","year":"2020","journal-title":"J Int Med Res."},{"key":"B5","doi-asserted-by":"crossref","first-page":"e240152","DOI":"10.1136\/bcr-2020-240152","article-title":"Pulmonary epithelioid haemangioendothelioma mimicking lung cancer.","volume":"14","author":"Oda","year":"2021","journal-title":"BMJ Case Rep."},{"key":"B6","first-page":"8048056","article-title":"Case of pulmonary epithelioid hemangioendothelioma with literature review.","volume":"2020","author":"Aung","year":"2020","journal-title":"Case Rep Oncol Med."},{"key":"B7","doi-asserted-by":"publisher","first-page":"6239","DOI":"10.2147\/CMAR.S310115","article-title":"Drug-eluting beads bronchial arterial chemoembolization in treating relapsed\/ refractory small cell lung cancer patients: results from a pilot study.","volume":"13","author":"Lin","year":"2021","journal-title":"Cancer Manag Res."},{"key":"B8","doi-asserted-by":"publisher","first-page":"1195","DOI":"10.1093\/jjco\/hyaa106","article-title":"Epithelioid hemangioendothelioma of the lung: CT findings and clinical course of 35 cases.","volume":"50","author":"Onishi","year":"2020","journal-title":"Jpn J Clin Oncol."},{"key":"B9","doi-asserted-by":"crossref","first-page":"59","DOI":"10.1016\/j.clinimag.2019.06.002","article-title":"Imaging findings in epithelioid hemangioendothelioma.","volume":"58","author":"Epelboym","year":"2019","journal-title":"Clin Imaging."},{"key":"B10","doi-asserted-by":"publisher","first-page":"778","DOI":"10.1016\/j.acra.2020.04.015","article-title":"Natural history of epithelioid hemangioendothelioma of the liver: CT findings of 15 cases.","volume":"28","author":"Onishi","year":"2021","journal-title":"Acad Radiol."},{"key":"B11","doi-asserted-by":"publisher","first-page":"661032","DOI":"10.3389\/fmed.2021.661032","article-title":"Case report: rare pulmonary sclerosing pneumocytoma: large, multiple, metastatic, and fatal.","volume":"8","author":"Zhang","year":"2021","journal-title":"Front Med."},{"key":"B12","doi-asserted-by":"publisher","first-page":"158","DOI":"10.1515\/med-2016-0025","article-title":"Role of FDG-PET scan in staging of pulmonary epithelioid hemangioendothelioma.","volume":"11","author":"Calabrese","year":"2016","journal-title":"Open Med."},{"key":"B13","doi-asserted-by":"publisher","first-page":"168","DOI":"10.1097\/LBR.0000000000000230","article-title":"Pulmonary epithelioid hemangioendothelioma diagnosed with endobronchial biopsies: a case report and literature review.","volume":"23","author":"Sakata","year":"2016","journal-title":"J Bronchol Interv Pulmonol."},{"key":"B14","doi-asserted-by":"publisher","first-page":"e004487","DOI":"10.1136\/jitc-2022-004487","article-title":"Pathological complete response to immune checkpoint inhibitor in patients with colorectal cancer liver metastases harboring POLE exonuclease domain mutation.","volume":"10","author":"Wen","year":"2022","journal-title":"J Immunother Cancer."},{"key":"B15","doi-asserted-by":"publisher","first-page":"e2100267","DOI":"10.1200\/PO.21.00267","article-title":"Clinical and molecular characterization of POLE mutations as predictive biomarkers of response to immune checkpoint inhibitors in advanced cancers.","volume":"1","author":"Garmezy","year":"2022","journal-title":"JCO Precis Oncol."},{"key":"B16","doi-asserted-by":"publisher","first-page":"e826","DOI":"10.1097\/MPH.0000000000001643","article-title":"Retrospective review of the use of sirolimus for pediatric patients with epithelioid hemangioendothelioma.","volume":"42","author":"Engel","year":"2020","journal-title":"J Pediatr Hematol."}],"container-title":["Frontiers in Medicine"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fmed.2022.1066870\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,12,15]],"date-time":"2022-12-15T06:49:04Z","timestamp":1671086944000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fmed.2022.1066870\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,12,15]]},"references-count":16,"alternative-id":["10.3389\/fmed.2022.1066870"],"URL":"https:\/\/doi.org\/10.3389\/fmed.2022.1066870","relation":{},"ISSN":["2296-858X"],"issn-type":[{"value":"2296-858X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,12,15]]},"article-number":"1066870"}}