This review highlights the diagnostic challenges and lack of standardized treatment guidelines for primary epithelioid hemangioendotheliomas of the liver.
No standardized EHL guidelines exist amid inconsistent outcomes; leaves open need for prospective trials to inform management.
Primary epithelioid hemangioendotheliomas of the liver (EHL) are rare tumors with a low incidence. The molecular background of EHL is still under investigation, with WWTR1-CAMPTA1 mutation may function as a tumor marker. Commonly, this tumor is misdiagnosed with angiosarcoma, cholangiocarcinomas, metastatic carcinoma, and hepatocellular carcinoma (sclerosing variant). Characteristic features on imaging modalities such as ultrasound, computed tomography, magnetic resonance imaging and positron emission tomography/computed tomography guide in diagnosis and staging. The "halo sign" and the "lollipop sign" on computed tomography and magnetic resonance imaging are described in the literature. Currently, there are no standardized guidelines for treating EHL with treatment options are broad including: chemotherapy, ablation, surgery and liver transplantation with inconsistent results.
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Virarkar et al. (2020) studied this question.
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