Angiosarcoma of the scalp and face: the Mayo 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.