Characteristics and Prognosis of Primary Head and Neck Angiosarcomas: A Surveillance, Epidemiology, and End Results Program (SEER) Analysis of 1250 Cases.

Head and neck angiosarcomas (HN-AS) are rare malignancies with a poor prognosis relative to other soft tissue sarcomas. To date, the HN-AS literature has been limited to short reports and single-institution experiences. This study evaluated patients registered with the Surveillance, Epidemiology, and End Results (SEER) program who had been diagnosed with a primary HN-AS. Predictors were drawn from demographic and baseline tumor characteristics. Outcomes were survival months and cause of death. Kaplan-Meier analyses were used to estimate overall (OS) and disease-specific survival (DSS) rates. Cox proportional hazards regression models were used for multivariate analyses. A total of 1250 patients (mean age 73.3 years) were identified, and nearly all lesions (93.5%) were cutaneous. Two- and 5-year OS rates were 47.3% (95% CI 44.3-50.3) and 26.5% (95% CI 23.7-29.3), while 2- and 5-year DSS rates were 66.6% (95% CI 63.6-69.6) and 48.3% (95% CI 44.5-52.1). In the univariate analyses, age, race, tumor grade, tumor size, AJCC stage, SEER historic stage, and surgery were significant predictors of both OS and DSS. Multivariate regression revealed that independent predictors of poor OS and DSS were older age [OS: HR 1.04 (95% CI 1.02-1.05), p < 0.01; DSS: HR 1.03 (95% CI 1.01-1.05), p < 0.01], increased tumor size [OS: HR 1.01 (95% CI 1.01-1.01), p < 0.01; DSS: HR 1.01 (95% CI 1.01-1.02), p < 0.01], and distant disease [OS: HR 2.97 (95% CI 1.65-5.34), p < 0.01; DSS: HR 4.99 (95% CI 2.50-9.98), p < 0.01]. Age, tumor size, and disease extent were determinants of HN-AS survival. When all other factors were controlled, lower histologic grade and surgery did not improve the risk of death.