Angiosarcoma of the scalp and face: the Mayo Clinic experience

• Published in: JAMA otolaryngology-- head & neck surgery Vol. 141; no. 4; p. 335
• Main Authors: Patel, Samir H, Hayden, Richard E, Hinni, Michael L, Wong, William W, Foote, Robert L, Milani, Shadi, Wu, Qing, Ko, Stephen J, Halyard, Michele Y
• Format: Journal Article
• Language: English
• Published: United States 01.04.2015
• Subjects: Age Factors; Aged; Combined Modality Therapy; Disease-Free Survival; Facial Neoplasms - mortality; Facial Neoplasms - pathology; Facial Neoplasms - therapy; Female; Head and Neck Neoplasms - mortality; Head and Neck Neoplasms - pathology; Head and Neck Neoplasms - therapy; Hemangiosarcoma - mortality; Hemangiosarcoma - pathology; Hemangiosarcoma - therapy; Humans; Male; Neoplasm Recurrence, Local - mortality; Neoplasm Recurrence, Local - pathology; Retrospective Studies; Scalp; Skin Neoplasms - mortality; Skin Neoplasms - pathology; Skin Neoplasms - therapy; Survival Rate; Treatment Outcome
• ISSN: 2168-619X
• DOI: 10.1001/jamaoto.2014.3584

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. 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. 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. Surgery, radiation therapy, and/or chemotherapy. Locoregional control (LRC), recurrence-free survival (RFS), and overall survival (OS). 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). 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.