An Aggressive Large Epithelioid Hemangioendothelioma of the Anterior Mediastinum in a Young Woman.
10.5090/kjtcs.2018.51.6.419
- Author:
Roman DUTTA
1
;
Harish PAL
;
Garima GARG
;
Sambit MOHANTY
Author Information
1. Department of Thoracic Surgery, Nayati Medicity, India.
- Publication Type:Case Report
- Keywords:
Hemangioendothelioma;
Mediastinum;
Vascular neoplasms
- MeSH:
Adolescent;
Brain;
Diagnosis;
Drug Therapy;
Female;
Hemangioendothelioma;
Hemangioendothelioma, Epithelioid*;
Humans;
Immunohistochemistry;
Incidence;
Liver;
Lung;
Mediastinum*;
Vascular Neoplasms
- From:The Korean Journal of Thoracic and Cardiovascular Surgery
2018;51(6):419-422
- CountryRepublic of Korea
- Language:English
-
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.
