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.

Angiosarcoma of the Thyroid and Regional Lymph Node Metastasis

“…The term angiosarcoma is commonly used for all sarcomas showing endothelial differentiation whether they originate in the vascular or lymphatic endothelium [9,10]. Angiosarcomas have a predilection for cutaneous sites, especially in the head and neck region of elderly male patients [11].…”

Treatment of cutaneous angiosarcoma of the scalp and face in the Chinese patients: local experience at a regional hospital in Hong Kong

“…Nonetheless, the results of RT were compromised when palliative cases were incorporated: only 12% of patients survived more than 5 years in one study. 14 Our patients were older (median age, 81 years) than those reported in the literature (median age, 63-77 years) [ Table 3]. The prognostic significance of age on the outcome of HNCAS is controversial.…”

Excisional surgery of periocular angiosarcoma

“…Chemotherapy has been proven of very limited benefit. [3][4][5] The most common features are bruise-like macules, plaques and nodules or ulceration. It remains unclear whether these tumours arise de novo, although some cases are reported in which angiosarcoma developed in postradiation tissue or after chronic lymphedema.…”

“…It remains unclear whether these tumours arise de novo, although some cases are reported in which angiosarcoma developed in postradiation tissue or after chronic lymphedema. [1][2][3][4][5] Furthermore, angiosarcoma can present as rosacea clinically 6,7 and Gallardo et al 8 have described a patient in whom an angiosarcoma arose on a rhinophyma. In our patient with periocular angiosarcoma, a rhinophyma was present, but histopathology of the involved nasal skin showed no features suggestive of angiosarcoma.…”

“…[1][2][3] This tumour carries a poor prognosis despite treatment. [3][4][5] We present a patient with periocular angiosarcoma treated with wide, but eye-sparing, surgical excision and eyelid reconstruction. During 3 years of follow-up, he has remained disease-free.…”