Angiosarcoma of the head and neck

Background: Angiosarcoma (AS) is an uncommon, highly aggressive tumor with a poor prognosis. Methods: To study the impact of various treatment modalities, namely surgery, radiation, and chemotherapy, we reviewed our experience with AS of the head and neck. Results: From 1978 through 1992, we treated 13 men and 5 women with AS (median age 67 years). Sixteen tumors occurred on the scalp and face and 2 in the oropharynx. Two patients presented with cervical metastases, and a third had subsequent nodal involvement. Primary surgery was used in 9 patients, including 1 who received adjunctive systemic doxorubicin hydrochloride, and 2 who received adjunctive radiotherapy. The tumors of 9 patients were unresectable: 4 were treated with intra-arterial doxorubicin hydrochloride; and 5, with systemic doxorubicin hydrochloride. Twelve patients (67%) died of disease an average of 25 months after diagnosis. Overall 5-year survival was 33%, but only 20% of the patients were disease free. Size of the tumor was an important predictor of survival, as all patients with a lesion >10 cm died of disease, compared with 67% with a lesion <10 cm. Four of 6 patients treated with wide local excision for lesions <10 cm survived 5 years. Conclusions: We recommend surgery for resectable lesions with postoperative radiation for unsatisfactory margins, large tumor size, deep extension, and multicentricity. Elective treatment of the neck does not appear warranted.