Successful Management of Pancreatic Kaposiform Hemangioendothelioma With Sirolimus: Case Report and Literature Review
Kaposiform hemangioendothelioma (KHE) is a rare vascular tumor with an estimated prevalence of 0.91 case per 100,000 children. Kaposiform hemangioendothelioma frequently affects the dermis and subcutis. It has intermediate malignant and locally aggressive characteristics.1 The mortality risk of KHE is often related to Kasabach-Merritt phenomenon (KMP), which develops in 75% of KHE cases.1 Kasabach-Merritt phenomenon is characterized by severe thrombocytopenia, microangiopathic anemia, and hypofibrinogenemia. Retroperitoneal KHE had higher incidence of KMP compared with superficial lesions, which means a higher mortality rate in patients with retroperitoneal KHE.1 Although various medications, including steroids, vincristine, interferon α, propranolol, and sirolimus, either alone or in combination, have been used for the treatment of KHE, the optimal therapeutic regimen is still unknown. Given the challenging diagnosis and management for retroperitoneal KHE, we report a case of a 16-month-old boy with a pancreatic KHE and review the literature of retroperitoneal KHE.