Abstract：
Background: Dozens of paraneoplastic syndromes affect the visual system ranging from conjunctival pemphigoid to encephalopathy of the occipital cortex. The most profiled ocular syndromes are bilateral diffuse uveal melanocytic proliferation (BDUMP) and the autoimmune retinopathies.Purpose: To review the critical features of these 2 entities then concentrate on advancements in treatment made within the last 10 years.Study Design: Literature review with structured data abstraction.Results: Major insights into pathogenesis have been wanting. Plasmapheresis appears to improve vision in a substantial proportion of patients with BDUMP. The number of clinical variables that influence visual outcome in paraneoplastic retinopathies combined with the variety of local and systemic treatment options makes interpretation of clinical effectiveness difficult.Conclusions: The rarity of these disorders makes randomized clinical trials unlikely. It may be time for a clinical professional organization to use a modified Delphi method to establish a consensus algorithm for the diagnosis and management of retinal paraneoplastic syndromes to augment clinical communications and clinical trials.
摘要：
背景：数十种副肿瘤综合征会影响视觉系统，从结膜类天疱疮到枕叶皮质脑病。最常见的眼部综合征是双侧弥漫性葡萄膜黑素细胞增生（BDUMP）和自身免疫性视网膜病。目的：回顾这两个实体的关键特征，然后专注于过去10年中的治疗进展。研究设计：结构化数据抽象的文献综述。结果：对发病机制的主要见解一直缺乏。血浆置换似乎可以改善大部分BDUMP患者的视力。影响副肿瘤性视网膜病变的视觉结果的临床变量的数量以及各种局部和全身治疗选择使得临床有效性的解释变得困难。结论：这些疾病的罕见性使随机临床试验不太可能。临床专业组织可能是时候使用改良的Delphi方法建立共识算法来诊断和管理视网膜副肿瘤综合征，以增强临床沟通和临床试验。
