Sirolimus therapy for refractory kaposiform hemangioendothelioma with Kasabach-Merritt phenomenon: a case report
Case-based review, 51 - 55Tag this article
Kaposiform hemangioendothelioma (KHE) is a rare vascular tumor, frequently associated with Kasabach-Merritt phenomenon. KHE has a high mortality rate as a consequence of haemorrhagic complications and the compression of vital structures. Due to its rarity, no standard guidelines have been established for the treatment of the disease. We report the case of an unresectable lower limb and a retroperitoneal vascular tumor resistant to conventional medical therapy (steroids, propanolol and vincristine). Following multimodal therapy, sirolimus was initiated, during a 24-months period, with a substantial decrease in tumor size and a sustained platelet count of >50x109/L. This case highlights the diagnostic and therapeutic challenges of this rare entity, supporting and emphasizing the role of sirolimus as a favourable therapeutic approach.
KEY WORDS: kaposiform haemangioendothelioma; Kasabach-Merritt phenomenon; consumptive coagulopathy; sirolimus.