Clinical features of malignant haemangiopericytomas and haemangioendotheliosarcomas

The clinical features of 23 patients with malignant vascular tumours, referred for treatment in the period 1968-85, are described. The tumours consisted of 15 haemangioendotheliosarcomas (angiosarcomas) and 8 malignant haemangiopericytomas. Ten haemangioendotheliosarcomas were located in skin or mucous membranes and 5 in viscera and deep soft tissues. None of the 8 haemangiopericytomas were superficially located. One cutaneous haemangioendotheliosarcoma arose in a lymphoedematous leg, while 4 others occurred in previously irradiated areas. There was one case of a Thorotrast-induced hepatic haemangioendotheliosarcoma. No predisposing factors could be demonstrated in the cases of malignant haemangiopericytoma. Median survival was 11 months for the haemangioendotheliosarcomas and 19 months for the haemangiopericytomas. Patients who were operable at the time of admission had a longer median survival (37 months) than those who presented with advanced disease (5 months). Local control was obtained by surgery in 3 cases and by radiation therapy in 2. No complete responses were seen on chemotherapeutic regimens.