Vincristine and corticosteroids as first-line treatment of Kasabach-Merritt syndrome in kaposiform hemangioendothelioma.
Kasabach-Merritt syndrome-associated kaposiform hemangioendothelioma successfully treated with cyclophosphamide, vincristine, and actinomycin D.
3-7] Several retrospective studies on patients who presented with Kasabach-Merritt syndrome have revealed that what was originally diagnosed as a capillary hemangioma may often be better described as a Kaposiform hemangioendothelioma.
According to our review of the literature, the histopathology of Kaposiform hemangioendothelioma in the thymus has not been described previously.
The pathogenesis of Kaposiform hemangioendothelioma is unknown.
First, the Kaposiform hemangioendothelioma tends to be diffusely infiltrative, whereas the hemangioma tends to be circumscribed.
Typically, high mitotic rate and nuclear atypia are not features of Kaposiform hemangioendothelioma.
Although both entities (Kaposiform hemangioendothelioma and Kaposi sarcoma) have spindled cells with slitlike lumina, hyaline globules, hemosiderin deposition, and endothelial antigen expression, the Kaposiform hemangioendothelioma also has areas of epithelioid endothelium, often in glomeruloid nests.