Shields Jerry A, Demirci Hakan, Mashayekhi Arman, Shields Carol L
Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania 19107, USA.
Ophthalmology. 2004 Sep;111(9):1739-46. doi: 10.1016/j.ophtha.2004.02.016.
To report clinical variations and the natural course of melanocytoma of the optic disc.
A retrospective study was conducted on a consecutive series of patients with melanocytoma of the optic disc who were evaluated at the Ocular Oncology Service at Wills Eye Hospital. Demographic information and variations in appearance and size of the tumor and local ocular complications were noted. Frequency of tumor growth and malignant transformation was recorded. Clinical factors, including patient data and tumor features, were analyzed for their impact on visual acuity loss of 2 or more Snellen lines and the tumor growth, using Cox proportional hazards regression models. Kaplan-Meier survival estimates of probability of visual acuity loss of 2 or more Snellen lines and probability of tumor growth were performed as a function of time from the initial examination.
Visual acuity loss of 2 or more Snellen lines, tumor growth, and malignant transformation of the tumor.
There were 115 patients (116 eyes) with melanocytoma of the optic disc. The mean age at diagnosis was 50 years; 38% of patients were male and 62% of patients were female; 65% of patients were white, 29% of patients were African American, and 6% of patients were Asian, Hispanic, Indian, or Arabic. The lesion was unilateral in 99% of patients. Visual symptoms that seemed to be related to the melanocytoma were present in 24% of patients, and an afferent pupillary defect was noted in 9% of patients. Associated ocular abnormalities included ocular melanocytosis (8%), racial melanosis (7%), optic nerve hypoplasia (2%), and retinitis pigmentosa (1%). The melanocytoma was dark brown to black in 100% of patients. The mean tumor diameter was 2 mm and the mean thickness was 1 mm. Associated findings included a choroidal component (54%), retinal component (30%), optic disc edema (25%), retinal edema (16%), localized subretinal fluid (14%), retinal exudation (12%), retinal hemorrhage (5%), vitreous seeds (4%), and retinal vein obstruction (3%). Fluorescein and indocyanine green angiography typically showed persistent hypofluorescence of the lesion. Using Kaplan-Meier survival curves, related visual loss occurred in 18% of patients by 10 years, and minor tumor enlargement occurred in 11% of patients by 5 years and in 32% of patients by 10 years. Malignant transformation was documented in 2 patients (2%).
Although melanocytoma of the optic disc generally is considered to be a benign, stationary lesion, it can produce several local complications, can cause visual loss, can grow slowly, and, rarely, can undergo malignant transformation into melanoma. Patients with optic disc melanocytoma should undergo periodic ocular examination.
报告视盘黑色素细胞瘤的临床变异及自然病程。
对在威尔斯眼科医院眼肿瘤科接受评估的一系列连续的视盘黑色素细胞瘤患者进行回顾性研究。记录人口统计学信息、肿瘤外观和大小的变异以及局部眼部并发症。记录肿瘤生长和恶变的频率。使用Cox比例风险回归模型分析包括患者数据和肿瘤特征在内的临床因素对视力丧失2行或更多Snellen视力表行数以及肿瘤生长的影响。根据初次检查后的时间，对视力丧失2行或更多Snellen视力表行数的概率和肿瘤生长的概率进行Kaplan-Meier生存估计。
视力丧失2行或更多Snellen视力表行数、肿瘤生长以及肿瘤恶变。
共有115例患者（116只眼）患有视盘黑色素细胞瘤。诊断时的平均年龄为50岁；38%的患者为男性，62%的患者为女性；65%的患者为白人，29%的患者为非裔美国人，6%的患者为亚洲人、西班牙裔、印度人或阿拉伯人。99%的患者病变为单侧。24%的患者存在似乎与黑色素细胞瘤相关的视觉症状，9%的患者存在传入性瞳孔障碍。相关的眼部异常包括眼部黑变病（8%）、种族性黑变病（7%）、视神经发育不全（2%）和视网膜色素变性（1%）。100%的患者黑色素细胞瘤为深棕色至黑色。肿瘤平均直径为2mm，平均厚度为1mm。相关表现包括脉络膜成分（54%）、视网膜成分（30%）、视盘水肿（25%）、视网膜水肿（16%）、局限性视网膜下液（14%）、视网膜渗出（12%）、视网膜出血（5%）、玻璃体播散（4%）和视网膜静脉阻塞（3%）。荧光素和吲哚菁绿血管造影通常显示病变持续低荧光。使用Kaplan-Meier生存曲线，10年时18%的患者出现相关视力丧失，5年时11%的患者肿瘤轻度增大，10年时32%的患者肿瘤轻度增大。2例患者（2%）记录到恶变。
尽管视盘黑色素细胞瘤通常被认为是一种良性的静止性病变，但它可产生多种局部并发症，可导致视力丧失，可缓慢生长，且极少可恶变为黑色素瘤。视盘黑色素细胞瘤患者应定期进行眼部检查。
