Cutaneous malignant hemangioendothelioma: clinical and histopathological observations of nine patients and a review of the literature.

Nine patients with cutaneous malignant hemangioendothelioma (CMHE) were reported in Okinawa. All the patients were elderly, between 75 and 93 years of age. Four patients were males and five were female. The onset of the disease ranged from 1 to 9 months before the first visit. Eight patients had lesions on the scalp, and one, on the face and cheek. The lesions were in the form of exudative erythematous purpura, erythematous purpuric ulcers, and tumors. One patient developed a systematic metastasis involving the lungs, heart and intestine, and two patients had local metastasis to the cervical lymph nodes. Histopathologically, the tumor vessels were proliferated irregularly and showed anastomosis. The lumens were lined by large and atypical endothelial cells. Most of the specimens were infiltrated with large numbers of red blood cells. By electron microscope, Weibel-palade bodies were found inside the tumor cells located at the peripheral part of the lesion. The patients were treated by irradiation, IL-2 injection, and/or surgery. They were treated for 3 months to 2 years. Eight patients died between 4 to 24 months after the onset of disease and one has survived. The prognosis was poor.