A rare case of asymptomatic mediastinal epithelioid hemangioendothelioma with diffuse liver involvement in a patient on active surveillance

Introduction

Epithelioid hemangioendothelioma (EHE) is a locally aggressive vascular neoplasm with metastatic potential (Yakobson A, Case Rep Oncol. 2021 Mar 3;14(1):309–317). EHE has a prevalence of less than one case in 1 million individuals and accounts for approximately less than 1% of all vascular tumors. The mean patient age is 43 years old, with a slightly higher preponderance among female patients. The most common sites of involvement are the liver, bones, and lungs, but they can involve many other sites throughout the body (Sardaro A, Oncol Rev 2014; 8:259). The etiology of EHE remains unclear, although several clonal chromosomal abnormalities have been identified (Antonescu CR, Genes Chromosomes Cancer 2013; 52:775–784). Clinical and laboratory data are non-specific, and diagnosis is based primarily on radiological, histological, and immunohistochemical features. The prognosis of EHE differs greatly and no optimal treatment strategy has been established yet (Patrini D, Respir Med Case Rep. 2017 Jun 3;22:19–23).

Publication History

Received: 26 November 2024

Accepted after revision: 05 February 2025

Article published online:
21 March 2025