Korean J Thorac Cardiovasc Surg.  2018 Dec;51(6):419-422. 10.5090/kjtcs.2018.51.6.419.

An Aggressive Large Epithelioid Hemangioendothelioma of the Anterior Mediastinum in a Young Woman

• Dutta R
• Pal H
• Garg G
• Mohanty S

• KMID: 2433887
• DOI: http://doi.org/10.5090/kjtcs.2018.51.6.419

Abstract

Hemangioendothelioma is a rare vascular tumor with involvement of the liver, brain, long bones, and lung. Among the 6 histological subtypes, epithelioid hemangioendothelioma (EHE) is the most aggressive. Its occurrence in the mediastinum is quite rare, and very few cases have been documented. The reported cases in the literature have described difficulties in the preoperative diagnosis due to the unusual histological appearance of the tumor. Immunohistochemistry remains the mainstay for a definitive diagnosis. Due to its low incidence, there is no standard treatment for mediastinal EHE, but curative resection is the preferred treatment option where possible, with chemotherapy used as an adjuvant treatment or in cases of widespread inoperable disease. The present case study describes an aggressive EHE occurring in an 18-year-old woman in the anterior mediastinum.

Keyword

Hemangioendothelioma; Mediastinum; Vascular neoplasms

MeSH Terms

Adolescent
Brain
Diagnosis
Drug Therapy
Female
Hemangioendothelioma
Hemangioendothelioma, Epithelioid*
Humans
Immunohistochemistry
Incidence
Liver
Lung
Mediastinum*
Vascular Neoplasms