{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,27]],"date-time":"2026-10-27T03:14:55Z","timestamp":1793070895754,"version":"4.5.0"},"reference-count":53,"publisher":"Oxford University Press (OUP)","issue":"7","license":[{"start":{"date-parts":[[2024,3,30]],"date-time":"2024-03-30T00:00:00Z","timestamp":1711756800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2024,7,7]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>Epithelioid hemangioendothelioma (EHE) is a remarkably rare tumor arising from endothelial cells that is classified as a vascular tumor in the WHO classification. The tumor is predominantly characterized by the presence of fusion genes, such as WWTR1-CAMTA1 or YAP1-TFE3, with a minority of cases exhibiting other rare fusion genes. EHE exhibits a broad age of onset, typically presenting at ~50\u00a0years, but it is not uncommon in pediatric populations. It manifests in a variety of organs, including the liver, lung, soft tissue and bone. Initial multiple-organ involvement is also observed. The tumor\u2019s biological behavior and prognosis vary substantially based on the primary site of manifestation. From a therapeutic perspective, initial active surveillance might be considered in selected cases, although surgical intervention remains the mainstay of treatment, especially for localized single-organ involvement. Chemotherapy is administered to patients with progressive unresectable tumors. Recent advances in the biological analysis of EHE fusion genes have elucidated their diverse functions. Additionally, next-generation sequencing has facilitated the identification of other mutations beyond the fusion genes. These continuous efforts to understand the biology of the fusion genes themselves and\/or the dysregulated signaling by fusion genes are expected to lead to the development of novel therapeutic strategies for EHE. This article aims to provide a comprehensive review of EHE, encompassing its historical context, clinical manifestations, molecular biology and the current state of treatment.<\/jats:p>","DOI":"10.1093\/jjco\/hyae037","type":"journal-article","created":{"date-parts":[[2024,3,31]],"date-time":"2024-03-31T05:31:50Z","timestamp":1711863110000},"page":"739-747","source":"Crossref","is-referenced-by-count":29,"title":["Epithelioid hemangioendothelioma\u2014its history, clinical features, molecular biology and current therapy"],"prefix":"10.1093","volume":"54","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9014-6382","authenticated-orcid":false,"given":"Kenji","family":"Tsuchihashi","sequence":"first","affiliation":[{"name":"Department of Hematology , Oncology and Cardiovascular Medicine, , Fukuoka , Japan"},{"name":"Kyushu University Hospital , Oncology and Cardiovascular Medicine, , Fukuoka , Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eishi","family":"Baba","sequence":"additional","affiliation":[{"name":"Department of Oncology and Social Medicine, Kyushu University Graduate School of Medical Sciences , Fukuoka , Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2024,3,30]]},"reference":[{"key":"2024070811031363400_ref1","doi-asserted-by":"crossref","first-page":"163","DOI":"10.1002\/path.1711280308","article-title":"Histogenesis of the so-called intravascular bronchioloalveolar tumour","volume":"128","author":"Corrin","year":"1979","journal-title":"J Pathol"},{"key":"2024070811031363400_ref2","first-page":"174","article-title":"Angiogenic nature of the \u201cintravascular bronchioloalveolar tumor\u201d of the lung: an electron microscopic study","volume":"105","author":"Weldon-Linne","year":"1981","journal-title":"Arch Pathol Lab 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