Hémangioendothéliome kaposiforme congénital cervical compliqué d’un phénomène de Kasabach-Merritt

Kaposiform hemangioendothelioma is a rare vascular tumor affecting infants and young children. It is associated with high morbidity and mortality, particularly due to the significant risk of developing Kasabach-Merritt phenomenon. This syndrome is characterized by severe thrombocytopenia and consumptive coagulopathy. Its presence makes the therapeutic management of kaposiform hemangioendothelioma difficult. First-line treatment has not yet been standardized and has long been based on the combination of vincristine and corticosteroids. In recent years, sirolimus has emerged as a treatment for kaposiform hemangioendothelioma with proven efficacy, either alone or in combination with corticosteroids, particularly in severe forms associated with Kasabach-Merritt phenomenon. It is now considered one of the first-line treatments for kaposiform hemangioendothelioma, particularly because of its good tolerance at low doses in children. Surgical treatment should be discussed depending on the degree of infiltration of the tumor lesion and the risk of hemorrhage secondary to the procedure.