Kaposiform Hemangioendothelioma
Overview
This is a vascular tumor associated with the Kasabach-Merritt phenomenon (severe coagulopathy due to platelet trapping and spontaneous bleeding). These tumors are histopathologically distinct from the common hemangiomas of infancy.
Symptoms
* Abdominal mass * Ascites * Intestinal obstruction * Jaundice * Lump deep in soft tissue * Dark skin plaque
Diagnosis
The MRI findings of Kaposiform hemangioendothelioma also clearly differ from those of common infantile hemangioma. In contrast to common hemangioma, this neoplastic birthmark characteristically involves multiple tissue planes, with cutaneous thickening, subcutaneous stranding, and edema. Gradient-echo images may demonstrate hemosiderin. Superficial feeding and draining vessels are less frequent and less prominent than with common hemangioma. Destructive osseous changes in the adjacent bones are common in Kaposiform hemangioendothelioma, whereas only some remodeling occasionally occurs in infantile hemangiomas.
Treatment
Kaposiform hemangioendothelioma has a mortality rate of 24%, which is related to coagulopathy or complications of local tumor infiltration. The tumor is associated with extremely low platelet counts and markedly decreased fibrinogen levels. Patients with Kaposiform hemangioendothelioma need to be closely monitored for severe coagulopathy. Pharmacological treatment regimen is similar to that of infantile hemangioma depending on the severity of the condition. Other treatment alternatives for patients with kaposiform hemangioendothelioma are transcatheter embolization and surgical excision.