Key result
Hepatectomy and liver transplantation serve as first-line treatments for rare hepatic epithelioid hemangioendothelioma.
Why the study?
Hepatic epithelioid hemangioendothelioma is a rare vascular tumor with unclear etiology and clinical manifestations, requiring an updated summary of literature on its diagnosis and treatment.
Hepatectomy and liver transplantation remain the primary treatments for hepatic epithelioid hemangioendothelioma, a rare vascular tumor.
Supports surgical approaches for this rare tumor; leaves open prospective evaluation of systemic options.
With an estimated incidence of only 1-2 cases in every 1 million people, hepatic epithelioid hemangioendothelioma (HEHE) is a rare vascular endothelial cell tumor occurring in the liver and consisting of epithelioid and histiocyte-like vascular endothelial cells in mucus or a fibrotic matrix. HEHE is characterized as a low-to-moderate grade malignant tumor and is classified into three types: solitary, multiple, and diffuse. Both the etiology and characteristic clinical manifestations of HEHE are unclear. However, HEHE has a characteristic appearance on imaging including ultrasound, magnetic resonance imaging, and positron emission tomography/computerized tomography. Still, its diagnosis depends mainly on pathological findings, with immunohistochemical detection of endothelial markers cluster of differentiation 31 (CD31), CD34, CD10, vimentin, and factor VIII antigen as the basis of diagnosis. Hepatectomy and/or liver transplantation are the first choice for treatment, but various chemotherapeutic drugs are reportedly effective, providing a promising treatment option. In this review, we summarize the literature related to the diagnosis and treatment of HEHE, which provides future perspectives for the clinical management of HEHE.
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Kou et al. (2020) conducted a review in Hepatic epithelioid hemangioendothelioma (HEHE). Diagnosis and treatment of HEHE was evaluated. Hepatic epithelioid hemangioendothelioma is a rare vascular tumor diagnosed via pathology and immunohistochemistry, with hepatectomy and liver transplantation serving as first-line treatments.
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