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

Abstract

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.

Histologic and Immunopathologic Variability in Primary Intraoral Angiosarcoma: A Case Report and Review of the Literature

“…Primary intraoral angiosarcoma most commonly affects older individuals, but exceptionally may affect pediatric patients. The peak age incidence for primary intraoral angiosarcoma is similar to that reported for soft tissue angiosarcomas, with most cases occurring in the 7th decade [15], and is slightly younger than the peak incidence from all head and neck subsites [35]. In pediatric patients, angiosarcoma is exceedingly rare, and most occur in the heart/pericardium [36].…”

“…Primary intraoral angiosarcomas are rare, and in the head and neck region most commonly involve the scalp. A recent analysis of 1250 angiosarcomas of the head and neck, found the tongue to be the second most common, non-cutaneous site for head and neck angiosarcoma after the facial bones, with 14% (11/80) of cases occurring in this site [35]. The oral cavity can also be affected by metastatic angiosarcoma and a large series of intraoral and salivary gland angiosarcomas reported 7 metastatic lesions, of which 5 arose from a skin primary in the head and neck [4].…”

“…Surgical excision is the most common treatment modality for all angiosarcomas, including those arising intraorally [4,58], with some patients also receiving radiation treatment or chemotherapy. In general, as a stand-alone treatment, radiation is reported to be less effective than surgery, and chemotherapy is recommended for local disease not amenable to surgery, or metastatic disease [35,59,60].…”

“…Angiosarcoma is an aggressive neoplasm that is generally associated with a poor prognosis [9]. Head and neck cases of angiosarcoma are no exception, and recently, Lee et al reported the 5-year overall survival for head and neck angiosarcomas to be 26.5% [35]. Fanburg-Smith et al, suggested that primary oral angiosarcomas may behave more favorably when compared to angiosarcomas of other sites.…”

Soft Tissue Special Issue: Perivascular and Vascular Tumors of the Head and Neck

“…Survival rates are generally poor, with a 5-year overall and disease specific survival of 26.5% and 48.3%, respectively. The traditional mainstay of AS treatment focuses on wide local excision with adjuvant radiotherapy [45,46]. Chemotherapy produces partial response in the metastatic setting.…”