Epithelioid Hemangioendothelioma as a Dangerous, Easy to Miss, and Nearly Impossible to Clinically Diagnose Condition: Case Report

TL;DR

This case report highlights the rare and difficult-to-diagnose vascular tumor epithelioid hemangioendothelioma (EHE), emphasizing the importance of early detection through biopsy and histopathological analysis.

Contribution

The novelty lies in presenting a rare clinical case of EHE with unique dermatological presentation and emphasizing key diagnostic markers for dermatologists.

Findings

A 24-year-old woman presented with periauricular erythematous papules, leading to the discovery of metastatic EHE.

Histology and immunohistochemistry confirmed the diagnosis of EHE despite its rarity and diagnostic challenges.

The case underscores the importance of recognizing EHE's clinical and histopathological features for timely diagnosis.

Abstract

Epithelioid hemangioendothelioma (EHE) is a rare vascular tumor with metastatic potential. EHE can have single- or multiorgan involvement, with presentations ranging from asymptomatic disease to pain and systemic symptoms. The extremely heterogeneous clinical presentation and disease progression complicates EHE diagnosis and management. We present the case of a 24-year-old woman with two periauricular erythematous papules, leading to the discovery of metastatic EHE through routine biopsy, despite a noncontributory medical history. Histology revealed the dermal proliferation of epithelioid cells and vacuoles containing red blood cells. Immunohistochemistry markers consistent with EHE solidified the diagnosis. Although extremely rare, prompt diagnosis of EHE is essential for informed decision-making and favorable outcomes. Key clinical and histopathological findings are highlighted to aid dermatologists in diagnosing and managing this uncommon condition.

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