Wesener Lambert, Hagelstein Victoria, Terheyden Patrick, Langan Ewan A
Clinic of Dermatology, Allergology and Venerology, University of Lübeck, 23562 Lübeck, Germany.
Department of Dermatological Sciences, University of Manchester, Oxford Rd., Manchester M13 9PL, UK.
J Clin Med. 2024 Aug 13;13(16):4741. doi: 10.3390/jcm13164741.
Despite the dramatic advances in the management of metastatic cutaneous melanoma, there remains no consensus-based, evidence-based strategy for the management of mucosal melanoma. The rare nature of the disease, its late clinical presentation, and distinct tumour biology all complicate efforts to optimise patient outcomes. To this end, we carried out a monocentric, retrospective analysis of all patients diagnosed with mucosal melanoma and treated between 2013 and 2021. Both tumour- and patient-specific characteristics were recorded, in addition to immune-related adverse events, in order to provide real-world data on disease progression, treatment efficacy, and the identification of prognostic markers. A total of 20 patients were identified (14 females and 6 males), with a mean age at diagnosis of 65.9 years. The median follow-up was 3.9 years (95% CI 1.4-6.4 years) from the initiation of systemic therapy. The median OS in the entire cohort was 1.9 years (95% CI 0.5-3.3 years). Performance status, sex, body mass index, and the presence of brain metastases were not associated with poorer outcomes. However, serum lactate dehydrogenase levels (LDH) ( = 0.04) and an NRAS mutation were markers of a poor prognosis ( = 0.004). There is a pressing need for real-world, prospective, and clinical trial data to inform the optimal management of mucosal melanoma, and data supporting the use of adjuvant and neo-adjuvant immunotherapy are currently lacking. However, an elevated LDH is a reliable, independent negative prognostic marker. Inter-disciplinary management remains essential in order to develop optimal treatment strategies.
尽管转移性皮肤黑色素瘤的治疗取得了显著进展,但对于黏膜黑色素瘤的治疗,仍没有基于共识和证据的策略。该疾病的罕见性、较晚的临床表现以及独特的肿瘤生物学特性,都使得优化患者治疗结局的努力变得复杂。为此,我们对2013年至2021年间所有诊断为黏膜黑色素瘤并接受治疗的患者进行了单中心回顾性分析。除了免疫相关不良事件外,还记录了肿瘤和患者的特定特征,以便提供关于疾病进展、治疗疗效及预后标志物识别的真实世界数据。共纳入20例患者(14例女性和6例男性),诊断时的平均年龄为65.9岁。从开始全身治疗起,中位随访时间为3.9年(95%CI 1.4 - 6.4年)。整个队列的中位总生存期为1.9年(95%CI 0.5 - 3.3年)。体能状态、性别、体重指数和脑转移的存在与较差的预后无关。然而,血清乳酸脱氢酶水平(LDH)(P = 0.04)和NRAS突变是预后不良 的标志物(P = 0.004)。迫切需要真实世界、前瞻性和临床试验数据来指导黏膜黑色素瘤的最佳治疗,目前缺乏支持辅助和新辅助免疫治疗应用的数据。然而,LDH升高是一个可靠的、独立的负面预后标志物。跨学科管理对于制定最佳治疗策略仍然至关重要。