Survival Outcomes in Primary Angiosarcoma of the Head and Neck: A Systematic Review and Meta-Analysis

Abstract

<h3>Purpose/Objective(s)</h3> Angiosarcoma of the head and neck (ASHN) is a rare entity and confers substantial morbidity and mortality. Yet, the optimal management of ASHN remains unclear. This study aimed to describe the epidemiology of ASHN and to identify the most favorable treatment approach. <h3>Materials/Methods</h3> We performed a systematic review based on the Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) guidelines, using the PubMed (Medline), EMBASE, and Cochrane Library databases, queried from 1990 until present. Articles in the English language reporting on survival outcomes of adult primary ASHN treated with curative-intent, were included. All estimates were weighted based on sample size. Analysis of variance (ANOVA) and two-sample t-tests were used as appropriate. <h3>Results</h3> A total of 3652 studies were identified, with 14 articles reporting on 2265 ASHN patients, meeting inclusion criteria. Mean ± SD age was 70.6 ± 7.7 years with 1621 (66.6%) men and 812 (33.4%) women. ASHN involved the scalp (n = 176, 57.9%) and the face (n = 128, 42.1%). 249 patients had early stage I-II disease (39.6%) whereas 379 had late stage III-IV disease (60.4%). Most (n = 529, 45.6%) received surgery and radiotherapy (RT), 305 (26.3%) received surgery alone, 210 (18.1%) received definitive RT/chemoradiotherapy (CRT), 75 (6.5%) received surgery and CRT, and 33 (2.8%) received surgery and chemotherapy. Negative margins were achieved in 471 (55.9%) whereas 371 (44.1%) had positive margins. Mean ± SD follow-up was 41.7 ± 15.4 months. Weighted mean, 1-, 5-, and 10-year overall survival (OS) were 26.9 months, 67.3%, 30.6%, and 20.8% respectively. Mean and 5-year disease-specific survival (DSS) were 72.9 months and 50.3% respectively. Mean ± SD local recurrence rate (LRR) was 32.1 ± 11.7%. Median RT dose delivered was 60 Gy (interquartile range: 60-70). Patients who received surgery had a significantly higher mean OS (34.9 vs. 18.7 months, <i>P</i> = 0.04) and 5-year OS (30.1 vs. 14.2%, <i>P</i> = 0.01) compared with those who did not receive surgery. There was no significant difference in mean OS for receiving adjuvant chemotherapy (<i>P</i> = 0.99) or adjuvant RT (<i>P</i> = 0.51). <h3>Conclusion</h3> In the largest ASHN study to date, definitive surgical resection was associated with an improvement in OS. Multimodality treatment did not confer an OS benefit. Randomized trials are needed to establish the optimal treatment approach for ASHN. This study was registered with PROSPERO (CRD42021220970).