Survival outcomes in primary head and neck adult sarcoma: A systematic review and meta-analysis.
Authors: Zayed Sondos , Lin Cindy , Boldt Gabriel , Lang Pencilla , Read Nancy , Venkatesan Varagur , Sathya Jinka , Kevin Fung , MacNeil Danielle , Mendez Adrian , Yoo John , Warner Andrew , Nichols Anthony C. , Palma David A. 
Published: June 2021

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Background: Head and neck sarcomas (HNS) are rare entities and confer substantial morbidity and mortality. Yet, the optimal management of HNS remains unclear. This study aimed to describe the epidemiology of HNS and to identify the most favorable treatment approach. Methods: We performed a systematic review and meta-analysis 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 HNS patients 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. Meta-analyses were performed using random effects models. This study was registered with PROSPERO, CRD42021220970. Results: A total of 3652 articles were identified, with 42 articles reporting on 21228 patients, meeting inclusion criteria. Mean ± SD age was 56.7 ± 14.6 years with 14170 (67.0%) men and 6991 (33.0%) women. The most common locations included skin and soft tissues (n = 12749, 63.3%), bones of skull and face (n = 2256, 11.2%), and oral cavity (n = 1775, 8.8%). The most common histologies included undifferentiated pleomorphic sarcoma (n = 5065, 24.8%), osteosarcoma (n = 2578, 12.6%), Kaposi sarcoma (n = 2316, 11.3%), chondrosarcoma (n = 2141, 10.5%), and hemangiosarcoma (n = 2072, 10.1%). 5459 patients had early stage I-II disease (76.9%) whereas 1643 had late stage III-IV disease (23.1%). Most received surgery alone (n = 10968, 61.0%), 3917 (21.8%) received surgery and radiotherapy (RT), 2173 (12.1%) received definitive RT/chemoradiotherapy (CRT), 811 (4.5%) received surgery and CRT, and 98 (0.5%) received surgery and chemotherapy. Negative margins were achieved in 6081 (76.5%). Mean ± SD follow-up was 55.3 ± 42.8 months. Weighted mean, 2-, 5-, and 10-year overall survival (OS) were 78.5 months, 75.9%, 63.2%, and 54.9% respectively. There was no significant difference in mean OS (P = 0.674) or 5-year OS (P = 0.965) between patients who received surgery alone, multimodality treatment with surgery and RT/CRT, or definitive RT/CRT. Mean ± SD 5-year OS was significantly higher with negative margins (62.7 ± 20.8%) compared with positive margins (22.7 ± 19.1%; P = 0.001). Mean ± SD local recurrence rate (LRR) was 32.0 ± 13.0%. LRRs were 41.8% for definitive RT/CRT, 39.3% for surgery and CRT, 33.6% for surgery alone, 24.7% for surgery and chemotherapy, and 20.1% for surgery and RT (P = 0.126). Conclusions: In the largest HNS study to date, negative margins were associated with an improvement in OS. Multimodality treatment did not confer an OS benefit. Definitive RT/CRT may be associated with a higher LRR. Randomized trials are needed to establish the optimal treatment approach for HNS.