Vanderbilt University School of Medicine, Nashville, TN, USA.
Department of Medicine, Vanderbilt University Medical Center and Vanderbilt Ingram Cancer Center, Nashville TN, USA.
Oncologist. 2022 Aug 5;27(8):703-710. doi: 10.1093/oncolo/oyac091.
Acral and mucosal melanomas (MM) are rare subtypes of melanoma that are biologically and clinically distinct from cutaneous melanoma. Despite the progress in the treatment of cutaneous melanomas with the development of targeted and immune therapies, the therapeutic options for these less common subtypes remain limited. Difficulties in early diagnosis, the aggressive nature of the disease, and the frequently occult sites of origin have also contributed to the poor prognosis associated with acral and MM, with substantially worse long-term prognosis. The rarity of these subtypes has posed significant barriers to better understanding their biological features and investigating novel therapies. Consequently, establishing standardized treatment guidelines has been a challenge. In this review, we provide a brief overview of the current knowledge regarding acral and MM, focusing on their epidemiology, genetic backgrounds, and unique clinical characteristics. Further discussion centers around the management of primary and advanced disease and the role of emerging targeted and immune therapies for these subtypes, specifically focusing on issues relevant to medical oncologists.
肢端和黏膜黑色素瘤(MM)是黑色素瘤的罕见亚型,在生物学和临床方面有别于皮肤黑色素瘤。尽管随着靶向和免疫治疗的发展,皮肤黑色素瘤的治疗取得了进展,但这些不太常见的亚型的治疗选择仍然有限。早期诊断困难、疾病的侵袭性以及起源部位通常隐匿,这些因素也导致了与肢端和 MM 相关的不良预后,其长期预后明显更差。这些亚型的罕见性给更好地了解其生物学特征和研究新疗法带来了重大障碍。因此,制定标准化的治疗指南一直是一个挑战。在这篇综述中,我们简要概述了目前关于肢端和 MM 的知识,重点介绍了它们的流行病学、遗传背景和独特的临床特征。进一步的讨论集中在原发性和晚期疾病的管理以及新兴的针对这些亚型的靶向和免疫治疗的作用,特别关注与肿瘤内科医生相关的问题。