Treatment of primary intraocular uveal melanoma has developed considerably, its driver genes are largely unraveled, and the ways to assess its risk for metastases are very precise, being based on an international staging system and genetic data. Unfortunately, the risk of distant metastases, which emerge in approximately one half of all patients, is unaltered. Metastases are the leading single cause of death after uveal melanoma is diagnosed, yet no consensus exists regarding surveillance, staging, and treatment of disseminated disease, and survival has not improved until recently. The final frontier in conquering uveal melanoma lies in solving these issues to cure metastatic disease. Most studies on metastatic uveal melanoma are small, uncontrolled, retrospective, and do not report staging. Meta-analyses confirm a median overall survival of 10–13 months, and a cure rate that approaches nil, although survival exceeding 5 years is possible, estimated 2% either with first-line treatment or with best supportive care. Hepatic ultrasonography and magnetic resonance imaging as surveillance methods have a sensitivity of 95–100% and 83–100%, respectively, to detect metastases without radiation hazard according to prevailing evidence, but computed tomography is necessary for staging. No blood-based tests additional to liver function tests are generally accepted. Three validated staging systems predict, each in defined situations, overall survival after metastasis. Their essential components include measures of tumor burden, liver function, and performance status or metastasis free interval. Age and gender may additionally influence survival. Exceptional mutational events in metastases may make them susceptible to checkpoint inhibitors. In a large meta-analysis, surgical treatment was associated with 6 months longer median overall survival as compared to conventional chemotherapy and, recently, tebentafusp as first-line treatment at the first interim analysis of a randomized phase III trial likewise provided a 6 months longer median overall survival compared to investigator's choice, mostly pembrolizumab; these treatments currently apply to selected patients. Promoting dormancy of micrometastases, harmonizing surveillance protocols, promoting staging, identifying predictive factors, initiating controlled clinical trials, and standardizing reporting will be critical steppingstones in reaching the final frontier of curing metastatic uveal melanoma.

原发性眼内葡萄膜黑色素瘤的治疗取得了长足的进步，其驱动基因在很大程度上未解开，评估其转移风险的方法非常精确，基于国际分期系统和遗传数据。不幸的是，大约一半的患者出现远处转移的风险没有改变。转移是葡萄膜黑色素瘤确诊后导致死亡的主要单一原因，但对于播散性疾病的监测、分期和治疗尚无共识，直到最近才改善生存率。征服葡萄膜黑色素瘤的最后一个领域在于解决这些问题以治愈转移性疾病。大多数关于转移性葡萄膜黑色素瘤的研究都是小型的、不受控制的、回顾性的，并且没有报告分期。荟萃分析证实中位总生存期为 10-13 个月，治愈率接近零，尽管生存期可能超过 5 年，估计一线治疗或最佳支持治疗为 2%。根据现行证据，作为监测方法的肝超声和磁共振成像在检测无辐射危害的转移灶方面的灵敏度分别为 95-100% 和 83-100%，但计算机断层扫描对于分期是必要的。除了肝功能测试之外，一般不接受任何基于血液的测试。三个经过验证的分期系统分别在特定情况下预测转移后的总生存期。它们的基本组成部分包括肿瘤负荷、肝功能和体能状态或无转移间隔的测量。年龄和性别也可能影响生存。转移灶中的异常突变事件可能使它们易受检查点抑制剂的影响。在一项大型荟萃分析中，与传统化疗相比，手术治疗与中位总生存期延长 6 个月相关，最近，在一项随机 III 期试验的第一次中期分析中，替苯他福作为一线治疗同样提供了延长 6 个月与研究者选择的中位总生存期相比，主要是派姆单抗；这些治疗目前适用于选定的患者。促进微转移的休眠、协调监测方案、促进分期、确定预测因素、启动对照临床试验和标准化报告将是达到治愈转移性葡萄膜黑色素瘤最终前沿的关键踏脚石。