原发性眼眶黑色素瘤：临床特征、治疗及预后的系统评价。
完整摘要 · 中文译文
目的：评估经组织病理学证实的原发性眼眶黑色素瘤（POM）的临床特征、治疗及预后。 方法：按照PRISMA指南进行了一项系统综述，并在PROSPERO注册。纳入报告1例及以上经组织病理学证实的POM且具有可提取数据的研究；排除继发性眼眶受累和非原始报告。提取人口学资料、临床特征、治疗及预后数据。肿瘤被分为黑色素细胞增多症/痣相关肿瘤（MATs）或新生黑色素瘤。使用Kaplan-Meier方法分析局部复发、转移、总生存期（OS）和无复发生存期（RFS）的结局。 结果：共纳入112例患者，其中49例（44%）为新生POM，34例（30%）为MATs，其余为未分类。MATs患者年龄稍轻（40.1岁 vs. 47.9岁；P=0.078），症状持续时间较短（5.3个月 vs. 13.1个月；P=0.158）。47%的病例接受了保留眼球的手术，53%接受了眶内容剜除术，37%接受了辅助放疗。在平均26.9个月的随访中，局部复发发生率为21%，转移发生率为39%。5年OS估计值显示有利于MATs的非显著性趋势（74% vs. 44%；P=0.058），而黑色素细胞增多症相关肿瘤的无复发生存期更长（P=0.048）。辅助放疗与改善的生存期相关（HR=0.23；95% CI：0.08-0.66；P=0.006）。 结论：POM是一种罕见的恶性肿瘤。MATs可能代表一个独特的亚型，预后较好，尽管解释受到许多小型且异质性数据集的限制。在某些病例中，保留眼球的治疗似乎有价值。
完整摘要 · 英文原文
PURPOSE: To evaluate the clinical characteristics, management, and outcomes for histopathologically confirmed primary orbital melanoma (POM). METHODS: A systematic review was conducted in accordance with PRISMA guidelines and registered with PROSPERO. Studies reporting 1 or more patients with histopathologically confirmed POM and extractable data were included; secondary orbital involvement and nonoriginal reports were excluded. Data were extracted for demographics, clinical features, treatment, and outcomes. Tumors were classified as either melanocytosis/nevus-associated (MATs) or de novo melanomas. Outcomes for local recurrence, metastasis, overall survival (OS), and recurrence-free survival (RFS) were analyzed using Kaplan-Meier methods. RESULTS: A total of 112 patients were included, of whom 49 (44%) had POMs that arose de novo, 34 (30%) were MATs, and the remainder were unclassified. Patients with MATs were slightly younger (40.1 vs. 47.9 y; P=0.078) and had shorter symptom duration (5.3 vs. 13.1 mo; P=0.158). Globe-sparing surgery was performed in 47% of cases, exenteration in 53%, and adjunctive radiotherapy in 37%. At a mean follow-up of 26.9 months, local recurrence occurred in 21% and metastasis in 39%. Estimates of 5-year OS showed a nonsignificant trend that favored MATs (74% vs. 44%; P=0.058), whereas recurrence-free survival was longer with melanocytosis-associated tumors (P=0.048). Adjuvant radiotherapy was associated with improved survival (HR=0.23; 95% CI: 0.08-0.66; P=0.006). CONCLUSIONS: POM is a rare malignancy. MATs may represent a distinct subset with more favorable outcomes, although interpretation is limited by the many small and heterogeneous data sets. Globe-sparing management appears to be valuable in some cases.