Clinical, Histopathological, and Immunophenotypic Spectrum of Hepatic Epithelioid Hemangioendothelioma: Eight Years' Data of a Tertiary Care Center from North India.

Background/Aims: Epithelioid 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 diagnosed in our center. Methods: All cases of HEHE diagnosed in our institute in the last eight years (2016-2023) were reviewed (n = 8; 11 samples) (total 36 cases of EHE; 22.2%). The clinical features, radiology, histopathology, immunophenotype, molecular features, and treatment outcomes of all cases were evaluated. Results: The median age of presentation was 49.5 years with a female: male ratio of 7:1. Abdominal pain was the commonest presentation. Approximately two-thirds 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 fluorescence Conclusions: We describe the clinico-histopathological features and differential diagnosis of HEHE. This appears to be the largest case series of HEHE from India.