Diagnosis of Hepatic Epithelioid Hemangioendothelioma: Challenges and Current Approaches

• DOI: https://doi.org/10.22533/at.ed.15952825060813

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• Abstract:

  Hepatic epithelioid hemangioendothelioma (HEHE) is a rare malignant vascular neoplasm with unpredictable clinical behavior and variable morphology, whose incidence is extremely low. Its nonspecific clinical presentation and the absence of specific laboratory markers make diagnosis a major challenge, often leading to errors and delays in treatment. This study aims to critically review the current diagnostic approaches to HEHE, highlighting the main clinical, radiological, histopathological, and immunohistochemical findings.
  Through a systematic review of the recent literature, evidence was analyzed that reinforces the role of advanced imaging techniques—such as magnetic resonance imaging and PET/CT—and immunohistochemical analysis with endothelial markers (CD31, CD34, factor VIII, among others) in differentiating HEHE from other liver neoplasms. Despite diagnostic advances, early identification still depends on a high degree of clinical suspicion and multidisciplinary integration. The absence of standardized protocols and the rarity of the disease reinforce the need for further clinical studies and international collaboration.