BackgroundEpithelioid hemangioendothelioma (EHE) is an uncommon vascular tumor that commonly affects the liver. Hepatic EHE (HEHE) presents with variable clinical and histopathological features. We describe detailed clinico-histopathological features, differential diagnosis, and treatment outcomes of the cases of HEHE. Materials and methodsAll cases of HEHE diagnosed in our institute in the last eight years (2016-2023) were reviewed (n=8) (total 36 cases of EHE; 22.2%). The clinical features, radiology, histopathology, immunophenotype, molecular features, and treatment outcomes of all cases were evaluated. ResultsThe median age of presentation was 49.5 years with a female: male ratio of 7:1. Abdominal pain was the commonest presentation. Approximately two-third of the patients had multifocal lesions. Histopathology showed purely epithelioid, predominantly epithelioid, and predominantly spindle-cell morphology in 50%, 25%, and 25% respectively. All cases showed typical myxohyaline/ fibrous stroma and organized thrombi of the portal/ central veins. CD31 was the most commonly used immunostain with positivity in all cases. CAMTA1 break-apart FISH was positive in 75% of cases while none showed TFE3 immunopositivity. Chemotherapy was the most commonly employed therapy (n=5) followed by surgery (n=2). The median duration of follow-up was 26 months. Five patients were alive with disease (two patients ≥3 years), one patient died of sudden cardiac death, and two patients were lost to follow-up. Two patients developed metastatic disease at follow-up. ConclusionWe describe the clinico-histopathological features and differential diagnosis of HEHE. This appears to be the largest case series of HEHE from India.