Angiosarcoma of the face and scalp, prognosis and treatment

Abstract

72 patients with angiosarcoma (AS) of the face and scalp have been analyzed with respect of various prognostic factors and the effects of different treatment regimes. This disease predominantly occurs in the elderly (age range, 56-92 years), and affects men rather more frequently than women (men: 44, women: 28). The clinical presentation varied, and included bruise-like lesions, dusky plaques, chronic edema or cellulitis, ulcerated nodules, pyoderma, and infected conditions. Due to lack of clinical awareness and problems with histologic assessment, delays in diagnosis were frequent. The majority of the tumors arose in the upper part of the face or scalp. Less commonly, the central part of the face was affected while only three tumors developed in the mandibular region. Overall the prognosis was poor; one half of the patients died within 15 months of presentation. Only 12% of the patients survived 5 years or more. Patients with lesions that were less than 10 cm in diameter responded better to treatment and statistically survived longer than those with larger lesions, emphasizing the crucial importance of early diagnosis. There is some indication that men, younger patients, patients with lesions on the central part of the face survived longer, but this was not of statistical significance. The histologic differentiation of the tumors at presentation also was of doubtful prognostic significance. Despite the overall poor prognosis, radical radiotherapy (mostly wide-field electron-beam therapy) in seven patients resulted in the apparent eradication of the local skin disease and prolonged survival of the patients. Pulmonary metastases developed 10 years later in two of those patients, however, but the face and scalp remained tumor-free.