Case#21

History: An infant presented with a large soft tissue mass-like lesion in the thigh. Reportedly the previous surgical excision of the lesion had revealed histopathological diagnosis of kaposiform hemangioendothelioma. Steroid treatment was initiated; however, the patient returned with clinical signs of hemorrhage. Diagnostic work up revealed severe degree low platelets, consistent with Kasabach Merritt syndrome. Despite aggressive medical therapy, the patient required angiographic assessment, then transcatheter embolization. The patient recovered completely.  

DISCUSSION: 

Kaposiform hemangioendothelioma is associated with Kasabach Merritt syndrome in some patients and may require aggressive therapy. Although a surgical intervention is needed in some patient, some benefit significantly from transcatheter embolization. It is important to follow these patients closely for potential bleeding complications. 

This is an angiographic appearance of the lesion. The lesion is quite hypervascular with multiple small arterial feeders from iliac and femoral arteries. It is impostant to note that there are no arteriovenous shunts.