Epithelioid hemangioendothelioma: disease course in 3 cases
Epithelioid hemangioendothelioma is an uncommon vascular neoplasm, one of intermediate-grade malignancy. Cutaneous epithelioid hemangioendothelioma is rare and often associated with multiple site involvement. Three patients with cutaneous EHE without systemic involvement in one case, with skin, liver and lung tumoral lesions in two others cases are reported. One patient is in complete remission after one year. The second patient is stable without treatment after ten years. In the third patient, alpha interferon given for one year don't produce effective results and the lesions do not progress without treatment after eight years of follow-up. Cutaneous presentation of EHE is quite variable. After the diagnosis is done, systemic involvement must be detected specially in bone, liver or lung. Metastatic spread or mulitcentric origin of the tumor are a matter of controversy. The pathobiologic behavior of EHE is not clearly recognized. Little data are available regarding the results of treatment with alpha interferon.