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- W2766684082 abstract "SESSION TITLE: Disorders of the Mediastinum 1 SESSION TYPE: Affiliate Case Report Poster PRESENTED ON: Tuesday, October 31, 2017 at 01:30 PM - 02:30 PM INTRODUCTION: Epithelioid hemangioendothelioma is a rare tumor originating from vascular cell lines typically in soft tissue and pulmonary parenchyma. We present a rare case of a 59-year-old male with a posterior mediastinal mass that was diagnosed as a epithelioid hemangioendothelioma (EHE). CASE PRESENTATION: A 59-year-old male presents to clinic for a persistent cough. His original CT Chest showed focal opacities which were diagnosed as cryptogenic organizing pneumonia, but a posterior mediastinal lesion was seen. He was treated with steroids with resolution of symptoms. Follow up imaging showed a peristent posterior mediastinal mass, reportedly a teratoma. Cardiothoracic surgery performed surgical resection of the mass. Pathology revealed an epithelioid hemangioendothelioma. Follow up positron emission tomography scan showed successful removal of the tumor without evidence of metastasis. DISCUSSION: EHE was first described in 1975 by Dail and Liebow as pulmonary epithelial hemangioendothelioma. It is a rare vascular tumor originating from vascular endothelial cells, formerly known as intravascular bronchioloalveolar tumor. It occurs less than 1% of all vascular tumors. Only 248 cases have been reported involving pulmonary tissue as of 2014. Involvement of soft tissue, pulmonary, liver and rarely bone has been reported. It has a female predominance (4:1) within pulmonary parenchyma. Males tend to have pleural involvement. Usual age of diagnosis is 20-60, median age of 36. Histologic pattern is described as an invasive growth pattern composed of pleomorphic cells with rare multinucleated giant cells. The cell types include epithelioid or dendritic cells with large eosinophilic cytoplasm in a fibromyxoid stroma. It stains for CD-31, cytokeratin, Factor VIII, CD-34, CAMTA-1, and ERG. The prognosis varies, median survival of 4.6 years. Currently, there is no standardized treatment. CONCLUSIONS: We present a rare case of EHE involving the posterior mediastinum. This could have easily been misdiagnosed as a benign teratoma with the potential to spread. This tumor typically involves the liver and/or pulmonary parenchyma, affecting females aged 20-60. There is no accepted standard therapy, and median survival is approximately 5 years. Reference #1: Sardaro A, Bardoscia L, Petruzzelli MF, Portaluri M. Epithelioid Hemangioendothelioma: An Overview and Update on a Rare Vascular Tumor. Oncology Reviews. 2014;8(2):259. doi:10.4081/oncol.2014.259. DISCLOSURE: The following authors have nothing to disclose: Grant Pierson, Marcel Junqueira No Product/Research Disclosure Information" @default.
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- W2766684082 date "2017-10-01" @default.
- W2766684082 modified "2023-09-25" @default.
- W2766684082 title "Rare Presentation of Epithelioid Hemangioendothelioma as a Posterior Mediastinal Tumor" @default.
- W2766684082 doi "https://doi.org/10.1016/j.chest.2017.08.521" @default.
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