[Epithelioid hemangioendothelioma].

Area of Science:

• Oncology
• Vascular Biology
• Genetics

Background:

• Epithelioid hemangioendothelioma (EHE) is a rare, low-grade vascular sarcoma.
• EHE often presents with a specific chromosomal translocation, t(1;3)(p36;q23-25), leading to WWTR1/CAMTA1 fusion protein synthesis in 90% of cases.
• EHE can manifest as localized or multifocal disease.

Purpose of the Study:

• To detail the initial presentation, diagnostic criteria, and outcomes of epithelioid hemangioendothelioma (EHE).
• To outline current management strategies for localized and multifocal EHE.
• To emphasize the need for interdisciplinary expertise in diagnosing and managing EHE.

Main Methods:

• Review of clinical, radiological, and histological data for epithelioid hemangioendothelioma (EHE) cases.
• Analysis of the association between chromosomal translocation t(1;3)(p36;q23-25) and EHE.
• Evaluation of treatment outcomes for localized and multifocal EHE based on disease progression.

Main Results:

• Epithelioid hemangioendothelioma (EHE) diagnosis relies on specific radiological and histological criteria.
• The WWTR1/CAMTA1 fusion protein, resulting from translocation t(1;3)(p36;q23-25), is a hallmark of most EHE cases.
• Localized EHE is best managed with focal treatments, while multifocal EHE warrants watchful follow-up, with some cases showing indolent progression.

Conclusions:

• Epithelioid hemangioendothelioma (EHE) management is stratified by disease stage (localized vs. multifocal).
• Watchful follow-up is the primary strategy for multifocal EHE, acknowledging its potential for indolent behavior.
• Effective diagnosis and management of EHE necessitate specialized, interdisciplinary care from dedicated centers.