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Clinical characterization and long‐term outcomes in pediatric epithelioid hemangioendothelioma

There is a paucity of information about the clinical characteristics and long‐term outcomes of pediatric epithelioid hemangioendothelioma (EHE), a rare vascular neoplasm commonly presenting in adulthood. In our case series of 24 patients with EHE aged 2‐26 years, the majority presented with multi‐organ disease. Progression was seen in 63% of patients with a mean time to progression of 18.4 months (range: 0‐72). Three patients treated with sirolimus achieved stable disease or partial response for >2.5 years. Longitudinal prospective pediatric studies are needed to develop standardized approaches to surgical and medical management.