Kaposiform hemangioendothelioma: Diagnosis and treatment

Kaposiform hemangioendothelioma (KHE) is a rare borderline vascular tumor that occurs primarily during infancy and childhood. The tumor typically originates in the skin and exhibits invasive growth into deeper tissues, often manifesting as a firm, poorly defined purplish-red mass. Cases of KHE involving visceral organs, including the bone, retroperitoneum, or mediastinum, have also been reported; however, their clinical features are often nonspecific, which may lead to delayed diagnosis. Importantly, these tumors are frequently associated with Kasabach-Merritt phenomenon, a consumptive coagulopathy that represents a major cause of mortality in KHE. Furthermore, tumor location, size, and clinical response to pharmacotherapy are closely associated with patient prognosis. Therefore, early recognition and timely treatment of KHE are essential. This article aims to review the epidemiology, etiology, clinical manifestations, diagnosis, and management of KHE.