LAUSR

Introduction: Kaposiform hemangioendothelioma (KHE) is a rare and aggressive vascular tumor that can be associated with a consumptive coagulopathy and thrombocytopenia (Kasabach-Merritt phenomenon). Only one case of an intracardiac KHE has been reported which was treated with surgical excision and then expectant management. Case Presentation: We present a patient with an intracardiac KHE which presented as a large mass surrounding the atria, pulmonary veins, superior vena cava, and infiltrating the atrial septum with moderate compression of the superior vena cava and mild compression of the pulmonary veins. This tumor clinically presented as persistent tachypnea and was unresponsive to conventional therapy with vincristine and steroids but responded dramatically to Sirolimus with almost complete regression on follow-up. Conclusions: None of the current treatments for KHE, alone or in combination therapy have been found to be effective in a uniform or reproducible manner. Well designed, preferably randomized trials are required for a better understanding of the appropriate dosage and duration as well as response to treatment and a consensus of first and second line therapies.