目的:头颈部粘膜黑色素瘤(MMHN)很少见,黑素细胞起源的侵袭性肿瘤,仍未完全了解,预后不良,局部复发率和远处转移率高。最近的几项研究扩大了对MMHN的理解,我们对有关其流行病学的最新证据进行了审查,分期,和管理。
方法:对报告和讨论流行病学的同行评审文章进行了文献检索,分期,和MMHN的管理。PubMed,Medline,搜索Embase和Cochrane图书馆以识别相关出版物。
■MMHN仍然是一种罕见的疾病。当前MMHN的TNM分期系统提供的风险分层不足,并且考虑诸如基于列线图的替代分期模型可能是合理的。具有清晰组织学边缘的肿瘤切除术仍然是最佳治疗的基石。辅助放疗可能会改善局部控制,但似乎不会影响生存率。免疫检查点抑制剂和c-KIT抑制剂在晚期或不可切除的粘膜黑素瘤患者中显示出有希望的疗效。并保证进一步研究探索联合疗法的效用。它们作为辅助疗法的作用尚未确定。新辅助系统治疗的疗效还不明确,尽管早期结果表明,它可能会改善结局。
结论:对流行病学的新见解,MMHN的分期和治疗改变了这种罕见恶性肿瘤的治疗标准.尽管如此,正在进行的临床试验和未来的前瞻性研究的结果需要更好地了解这种侵袭性疾病并优化其治疗.
OBJECTIVE: Mucosal melanoma of the head and neck (MMHN) are rare, aggressive neoplasms of melanocyte origin that remain incompletely understood and have a poor prognosis, with high rates of locoregional recurrence and distant metastasis. Several recent studies having expanded understanding of MMHN, we undertook a
review of the latest evidence pertaining to its epidemiology, staging, and management.
METHODS: A literature search was conducted for peer-reviewed articles reporting and discussing the epidemiology, staging, and management of MMHN. PubMed, Medline, Embase and the Cochrane Library were searched to identify relevant publications.
UNASSIGNED: MMHN remains an uncommon disease. The current TNM staging system for MMHN provides inadequate risk stratification, and consideration of an alternative staging model such as one based on a nomogram may be justifiable. Tumour resection with clear histological margins remains the cornerstone of optimal treatment. Adjuvant radiotherapy may improve locoregional control but does not appear to affect survival. Immune checkpoint inhibitors and c-KIT inhibitors demonstrate promising efficacy in patients with advanced or unresectable mucosal melanomas, and warrant further research exploring the utility of combination therapies. Their roles as adjuvant therapies have not been determined. The efficacy of neoadjuvant systemic therapy is also not yet clear, although early results suggest that it may improve outcomes.
CONCLUSIONS: New insights into the epidemiology, staging and management of MMHN have transformed the standard of care for this rare malignancy. Nonetheless, the results of ongoing clinical trials and future prospective studies are required to better understand this aggressive disease and optimise its management.