Abstract:
Primary uveal melanoma is rare and affects approximately 8,000 persons per year worldwide. This malignancy can involve the iris, ciliary body, and choroid. Of these three structures, the iris is the least commonly affected site, representing only 4% of all uveal melanomas. Iris melanoma can arise from iris melanocytic nevus, iris melanocytosis, or de novo. In a longitudinal study of 1,611 patients with iris nevus, transformation into melanoma, using Kaplan-Meier estimates, was found in 2.6% by five years and in 4.1% by 10 years. The factors that predicted growth of iris melanocytic nevus into melanoma are denoted by a letter (ABCDEF) guide: A for age ≤40 years old at presentation (hazard ratio [HR] = 3, P = .01), B for blood (hyphema) (HR = 9, P < .0004), C for clock hour of tumor inferiorly (tumor location) (HR = 9, P = .03), D for diffuse flat tumor configuration (HR = 14, P = .02), E for ectropion uveae (HR = 4, P = .002), and F for feathery ill-defined margins (HR = 3, P = .02). At diagnosis, iris melanoma has a mean cross-sectional diameter of 5.5 mm and thickness of 2.1 mm, often with tumor seeding (28%) and secondary glaucoma (35%). We provide a comprehensive review of iris nevus and melanoma to explore relevant demographic and clinical data, risk factors for tumor growth, management, and prognosis, with the hope that clinicians will be more comfortable in understanding this rare malignant condition.
摘要:
原发性葡萄膜黑素瘤是罕见的,并且在全世界每年影响大约8,000人。这种恶性肿瘤可能涉及虹膜,睫状体,和脉络膜.在这三种结构中,虹膜是最不常见的部位,仅占所有葡萄膜黑色素瘤的4%。虹膜黑素瘤可以由虹膜黑素细胞痣引起,虹膜黑素细胞增多症,或者从头。在一项对1,611例虹膜痣患者的纵向研究中,转化为黑色素瘤,使用Kaplan-Meier估计,在5年内发现2.6%,在10年内发现4.1%。预测虹膜黑素细胞痣生长为黑色素瘤的因素被字母(ABCDEF)指南记住,该指南代表A年龄≤40岁(HR=3,p=0.01),B表示血液(前房积血)(HR=9,p<0.0004),C表示肿瘤下方的时钟小时(肿瘤位置)(HR=9,p=0.03),D表示弥漫性扁平肿瘤构型(HR=14,p=0.02),外翻为E(HR=4,p=0.002),和F为羽毛不明确的边缘(HR=3,p=0.02)。诊断时,虹膜黑色素瘤显示平均横截面直径为5.5mm,厚度为2.1mm的临床特征,常伴有肿瘤种植(28%)和继发性青光眼(35%)。在这里,我们提供了虹膜痣和黑色素瘤的全面综述,以探索相关的人口统计学和临床数据,肿瘤生长的危险因素,管理,和预后,希望临床医生能够更舒适地理解这种罕见的恶性肿瘤。