Lechner Matt, Takahashi Yoko, Turri-Zanoni Mario, Ferrari Marco, Liu Jacklyn, Counsell Nicholas, Mattavelli Davide, Rampinelli Vittorio, Vermi William, Lombardi Davide, Saade Rami, Park Ki Wan, Schartinger Volker H, Franchi Alessandro, Facco Carla, Sessa Fausto, Battocchio Simonetta, Fenton Tim R, Vaz Francis M, O'Flynn Paul, Howard David, Stimpson Paul, Wang Simon, Hannan S Alam, Unadkat Samit, Hughes Jonathan, Dwivedi Raghav, Forde Cillian T, Randhawa Premjit, Gane Simon, Joseph Jonathan, Andrews Peter J, Dave Manas, Fleming Jason C, Thomson David, Zhu Tianyu, Teschendorff Andrew, Royle Gary, Steele Christopher, Jimenez Joaquin E, Laco Jan, Wang Eric W, Snyderman Carl, Lacy Peter D, Woods Robbie, O'Neill James P, Saraswathula Anirudh, Kaur Raman Preet, Zhao Tianna, Ramanathan Murugappan, Gallia Gary L, London Nyall R, Le Quynh-Thu, West Robert B, Patel Zara M, Nayak Jayakar V, Hwang Peter H, Hermsen Mario, Llorente Jose, Facchetti Fabio, Nicolai Piero, Bossi Paolo, Castelnuovo Paolo, Jay Amrita, Carnell Dawn, Forster Martin D, Bell Diana M, Lund Valerie J, Hanna Ehab Y
UCL Cancer Institute, University College London, London, United Kingdom.
UCL Division of Surgery and Interventional Science, University College London, London, United Kingdom.
J Neurol Surg B Skull Base. 2022 Jul 10;84(4):307-319. doi: 10.1055/s-0042-1750178. eCollection 2023 Aug.
Sinonasal mucosal melanoma (SNMM) is an extremely rare and challenging sinonasal malignancy with a poor prognosis. Standard treatment involves complete surgical resection, but the role of adjuvant therapy remains unclear. Crucially, our understanding of its clinical presentation, course, and optimal treatment remains limited, and few advancements in improving its management have been made in the recent past. We conducted an international multicenter retrospective analysis of 505 SNMM cases from 11 institutions across the United States, United Kingdom, Ireland, and continental Europe. Data on clinical presentation, diagnosis, treatment, and clinical outcomes were assessed. One-, three-, and five-year recurrence-free and overall survival were 61.4, 30.6, and 22.0%, and 77.6, 49.2, and 38.3%, respectively. Compared with disease confined to the nasal cavity, sinus involvement confers significantly worse survival; based on this, further stratifying the T3 stage was highly prognostic ( < 0.001) with implications for a potential modification to the current TNM staging system. There was a statistically significant survival benefit for patients who received adjuvant radiotherapy, compared with those who underwent surgery alone (hazard ratio [HR] = 0.74, 95% confidence interval [CI]: 0.57-0.96, = 0.021). Immune checkpoint blockade for the management of recurrent or persistent disease, with or without distant metastasis, conferred longer survival (HR = 0.50, 95% CI: 0.25-1.00, = 0.036). We present findings from the largest cohort of SNMM reported to date. We demonstrate the potential utility of further stratifying the T3 stage by sinus involvement and present promising data on the benefit of immune checkpoint inhibitors for recurrent, persistent, or metastatic disease with implications for future clinical trials in this field.
鼻窦黏膜黑色素瘤（SNMM）是一种极其罕见且具有挑战性的鼻窦恶性肿瘤，预后较差。标准治疗包括完整的手术切除，但辅助治疗的作用仍不明确。至关重要的是，我们对其临床表现、病程及最佳治疗方法的了解仍然有限，且近期在改善其治疗方面进展甚微。
我们对来自美国、英国、爱尔兰及欧洲大陆11家机构的505例SNMM病例进行了一项国际多中心回顾性分析。评估了临床表现、诊断、治疗及临床结局的数据。
1年、3年和5年无复发生存率及总生存率分别为61.4%、30.6%和22.0%，以及77.6%、49.2%和38.3%。与局限于鼻腔的疾病相比，鼻窦受累的患者生存率显著更差；基于此，进一步对T3期进行分层具有高度预后价值（P＜0.001），这可能对当前的TNM分期系统进行潜在修改。与单纯接受手术的患者相比，接受辅助放疗的患者有统计学意义的生存获益（风险比[HR] = 0.74，95%置信区间[CI]：0.57 - 0.96，P = 0.021）。对于复发或持续性疾病（无论有无远处转移），免疫检查点阻断治疗可延长生存期（HR = 0.50，95% CI：0.25 - 1.00，P = 0.036）。
我们展示了迄今为止报告的最大规模SNMM队列的研究结果。我们证明了根据鼻窦受累情况进一步对T3期进行分层的潜在效用，并展示了免疫检查点抑制剂对复发、持续性或转移性疾病有益的有前景的数据，这对该领域未来的临床试验具有启示意义。
