The prognosis and treatment of angiosarcoma of the face and scalp

Angiosarcoma of the face and scalp (AS) is a rare but well-recognized vascular tumour, generally accepted to have a very poor prognosis. However, most previous studies have been limited because of small patient numbers.
We have collected clinical data on 71 patients with AS from the time of initial histological diagnosis. Information obtained included age at diagnosis, sex, race, clinical appearance and size of lesions, therapeutic procedures, clinical course, incidence of metastases, survival time and response to therapy.
All the patients were Caucasian with a mean age of 74 years (range 56–72 years) and a male to female ratio of 1:5:1. Tumours occurred predominantly on the upper face and scalp but male patients frequently presented with central facial lesions. Three main clinical patterns were confirmed: nodules, bruise-like induration and areas of ulceration with bleeding.
Most patients were treated by radiotherapy (64%) or by excision alone (10%). The remainder received no therapy or combined therapy. Radiotherapy-treated patients fell into two groups: group I (71%) had short survival times ( x= 13.5 months), large lesions (63% < 10 cm), and a high incidence of metastatic disease (50% of patients); group 2 patients (29%) survived longer ( x = 5.5 years) and had a low proportion of large tumours (8%) with a low incidence of metastatic disease (8%). Patients treated by excision alone had a high incidence (86%) of metastatic disease.
Our data suggests that AS remains a disease of poor prognosis in elderly patients with large lesions. However, small (< 5 cm) lesions have a far better prognosis and the use of radiotherapy may be associated with prolonged survival and a lower incidence of metastases than simple excision.