A comprehensive analysis of epithelioid hemangioendothelioma from a tertiary cancer center in India

Introduction Epithelioid hemangioendothelioma (EHE), a rare vascular malignancy is categorized under malignant vascular neoplasm by the International Society for the Study of Vascular Anomalies (ISSVA) classification and the World Health Organization classification of soft tissue tumors. It shows a significant heterogeneity in clinical presentation and prognosis. Aims and objectives: To study the clinicopathological features of reported cases of EHE. Materials and Methods Retrospective data of reported cases of EHE were retrieved from the electronic medical records (EMR) of the Oncopathology department (Januray2019- September 2024). Demographic data, site, focality, metastasis, immunohistochemical results, treatment and follow up data were recorded. Results 17 cases were retrieved. Sites included liver (n = 6), skeletal system (n = 6), breast (n = 1), stomach (n = 1), Right iliac fossa (n = 1), Pleura (n = 1) and lung (n = 1). Immunohistochemistry panel comprised of a varying combination of CD31, CD34, FLI-1, ERG, D2-40, AE1/AE3 and TFE3 which were positive in 94.11%, 90%,100%, 64.28%, 42.85%, 64.28%, 60% respectively apart from other markers administered according the differential diagnosis considered. A mean size of 5.975 cm was recorded. The liver tumors were all multifocal. Metastasis was seen in eight cases. Tumor was disposed in cords, nests and sheets with an epithelioid to spindled morphology. A myxohyaline background was observed in five cases. Mitotic count was variable and necrosis was noted in five. The treatment received or planned was a varying combination of resection, radiotherapy, and systemic chemotherapy. The follow-up period ranged from 2 months to 5 years with a mean follow-up of nine months. One patient died of hemorrhagic shock, another due to pneumonia and the third died of the disease. Conclusion Awareness of the entity with the use of a panel of vascular markers helps to reach a correct diagnosis. Given the rarity of the disease, no established standard treatment for EHE is available.