Angiosarcoma of the scalp and face: Themayo clinic experience

IMPORTANCE: The etiology and optimal treatment are unknown for angiosarcoma, an aggressive malignant tumor that affects vascular endothelial cells and can be mistaken for benign lesions such as hemangioma. OBJECTIVE: To determine the treatment outcomes of patients with angiosarcoma of the face or scalp treated with a combination of surgery, radiation therapy, and/or chemotherapy. DESIGN, SETTING, AND PARTICIPANTS: Retrospective study of 55 patients with angiosarcoma of the face or scalp treated between January 1, 1973, and December 31, 2012, at a tertiary-care academic medical institution. INTERVENTIONS Surgery, radiation therapy, and/or chemotherapy. MAIN OUTCOMES AND MEASURES: Locoregional control (LRC), recurrence-free survival (RFS), and overall survival (OS). RESULTS: Fifty-five patients had angiosarcoma localized to the face or scalp. Forty of these patients (73%) received a combination of surgery, radiation therapy, and/or chemotherapy. Eight patients (15%) were treated with surgery alone, 1 (2%) with radiation alone, 5 (9%) with chemotherapy alone, and 1 (2%) with observation alone. Median (range) follow-up for surviving patients was 25.2 (4.7-227.1) months. Five-year LRC, RFS, and OS (95%CI) were 18% (7%-32%), 16%(6%-31%), and 38%(21%-54%), respectively. Of 36 patients with failed treatment, 34 had failure in a local and/or regional site. On univariate analysis, the use of multimodality therapy (vs no multimodality therapy) was associated with higher 5-year LRC (95%CI) (20% [3%-37%] vs 11% [0%-29%]; P =.04), higher RFS (19% [2%-36%] vs 10% [0%-27%]; P =.02), and higher OS (46%[26%-66%] vs 16%[0%-43%]; P =.04). Age 70 years or older (vs <70 years) was associated with lower 5-year LRC (95%CI) (5%[0%-14%] vs 48%[23%-74%]; P =.02) and lower RFS (5%[0%-13%] vs 49%[24%-75%]; P =.04). Radiation therapy (vs no radiation therapy) was associated with higher 5-year LRC (95%CI) (20% [3%-36%] vs 12%[0%-32%]; P =.02) and higher RFS (19% [2%-35%] vs 12% [0%-31%]; P =.004). On multivariable analysis, age younger than 70 years (vs≥70 years) was associated with improved 5-year LRC (95%CI) (48%[23%-74%] vs 5%[0%-14%]; P =.03) and RFS (49% [24%-75%] vs 49% [24%-75%]; P =.04). CONCLUSIONS AND RELEVANCE: Multimodality therapy for angiosarcoma is associated with improved LRC, RFS, and OS. Younger patients with resectable disease undergoing multimodality therapy for angiosarcoma had the best clinical outcomes.