Abstract：
OBJECTIVE: The aim of this work is to comprehensively review and synthesize the literature related to sinonasal mucosal melanoma (SNMM) treatment with immunotherapy, including potentially targetable genetic mutations, survival outcomes, and adverse events.
METHODS: Embase, Cochrane, Scopus, and Web of Science.
METHODS: The study protocol was designed according to Preferred Reporting Items for Systematic Reviews and Meta-analysis statement. Databases were searched from inception through May 23, 2023.
RESULTS: A total of 42 studies met inclusion criteria. Twenty-four of the included studies reported genetic mutations for a combined 787 patients with SNMM. 8.1% (95% confidence interval, CI: 7.6-8.6), 18.9% (95% CI: 18.1-19.8), and 8.5% (95% CI: 8.1-9.0) of reported patients were positive for BRAF, NRAS, and KIT mutations, respectively. The presence of brisk tumor-infiltrating lymphocytes was associated with improved recurrence-free survival and overall survival (OS). Six studies reported a combined 5-year OS after adjuvant immunotherapy treatment of 42.6% (95% CI: 39.4-45.8). Thirteen studies encompassing 117 patients reported adjuvant or salvage immune checkpoint inhibitor (ICI) immunotherapy response rates: 40.2% (95% CI: 36.8-43.6) had a positive response (tumor volume reduction or resolution). Eleven studies reported direct comparisons between SNMM patients treated with or without immunotherapy; the majority (7/11) reported survival benefit for their entire cohort or select subgroups of SNMM patients. With the transition to modern ICIs, there is a stronger trend toward survival improvement with adjuvant ICI. Tumors with Ki67 <40% may respond better to ICI\'s.
CONCLUSIONS: ICI therapy can be an effective in select SNMM patients, especially those with advanced/metastatic disease.
METHODS: Embase, Cochrane, Scopus, and Web of Science.
METHODS: The study protocol was designed according to Preferred Reporting Items for Systematic Reviews and Meta-analysis statement. Databases were searched from inception through May 23, 2023.
RESULTS: A total of 42 studies met inclusion criteria. Twenty-four of the included studies reported genetic mutations for a combined 787 patients with SNMM. 8.1% (95% confidence interval, CI: 7.6-8.6), 18.9% (95% CI: 18.1-19.8), and 8.5% (95% CI: 8.1-9.0) of reported patients were positive for BRAF, NRAS, and KIT mutations, respectively. The presence of brisk tumor-infiltrating lymphocytes was associated with improved recurrence-free survival and overall survival (OS). Six studies reported a combined 5-year OS after adjuvant immunotherapy treatment of 42.6% (95% CI: 39.4-45.8). Thirteen studies encompassing 117 patients reported adjuvant or salvage immune checkpoint inhibitor (ICI) immunotherapy response rates: 40.2% (95% CI: 36.8-43.6) had a positive response (tumor volume reduction or resolution). Eleven studies reported direct comparisons between SNMM patients treated with or without immunotherapy; the majority (7/11) reported survival benefit for their entire cohort or select subgroups of SNMM patients. With the transition to modern ICIs, there is a stronger trend toward survival improvement with adjuvant ICI. Tumors with Ki67 <40% may respond better to ICI\'s.
CONCLUSIONS: ICI therapy can be an effective in select SNMM patients, especially those with advanced/metastatic disease.
摘要：
目的：这项工作的目的是全面回顾和综合与免疫治疗治疗鼻窦粘膜黑色素瘤（SNMM）有关的文献，包括潜在的靶向基因突变，生存结果，和不良事件。
方法：Embase，科克伦,Scopus,和WebofScience。
方法：根据系统评价和荟萃分析陈述的首选报告项目设计研究方案。从开始到2023年5月23日搜索数据库。
结果：共有42项研究符合纳入标准。纳入的研究中有24项报告了787名SNMM合并患者的基因突变。8.1%(95%置信区间，CI:7.6-8.6)，18.9%（95%CI：18.1-19.8），8.5%（95%CI：8.1-9.0）的报告患者BRAF阳性，NRAS,和KIT突变，分别。活跃的肿瘤浸润淋巴细胞的存在与无复发生存率和总生存率（OS）的改善有关。6项研究报告辅助免疫疗法治疗后5年OS为42.6%(95%CI:39.4-45.8)。包括117名患者的13项研究报告了辅助或挽救性免疫检查点抑制剂（ICI）免疫疗法的反应率：40.2％（95％CI：36.8-43.6）具有阳性反应（肿瘤体积减少或消退）。11项研究报告了接受或未接受免疫治疗的SNMM患者之间的直接比较；大多数（7/11）报告了其整个队列或选择的SNMM患者亚组的生存益处。随着向现代ICI的过渡，佐剂ICI有更强的生存改善趋势。Ki67<40%的肿瘤可能对ICI的反应更好。
结论：ICI治疗对特定SNMM患者有效，尤其是那些患有晚期/转移性疾病的患者。
方法：Embase，科克伦,Scopus,和WebofScience。
方法：根据系统评价和荟萃分析陈述的首选报告项目设计研究方案。从开始到2023年5月23日搜索数据库。
结果：共有42项研究符合纳入标准。纳入的研究中有24项报告了787名SNMM合并患者的基因突变。8.1%(95%置信区间，CI:7.6-8.6)，18.9%（95%CI：18.1-19.8），8.5%（95%CI：8.1-9.0）的报告患者BRAF阳性，NRAS,和KIT突变，分别。活跃的肿瘤浸润淋巴细胞的存在与无复发生存率和总生存率（OS）的改善有关。6项研究报告辅助免疫疗法治疗后5年OS为42.6%(95%CI:39.4-45.8)。包括117名患者的13项研究报告了辅助或挽救性免疫检查点抑制剂（ICI）免疫疗法的反应率：40.2％（95％CI：36.8-43.6）具有阳性反应（肿瘤体积减少或消退）。11项研究报告了接受或未接受免疫治疗的SNMM患者之间的直接比较；大多数（7/11）报告了其整个队列或选择的SNMM患者亚组的生存益处。随着向现代ICI的过渡，佐剂ICI有更强的生存改善趋势。Ki67<40%的肿瘤可能对ICI的反应更好。
结论：ICI治疗对特定SNMM患者有效，尤其是那些患有晚期/转移性疾病的患者。
