Luo Jing-Yi, Yu Shi-Tong, Xu Xiao-Yu, Lin Xian-Xuan, Luo Rong-Jiang, Long Chong-De
State Key Laboratory of Ophthalmology, Zhongshan Ophthalmic Center, Sun Yat-Sen University, Guangdong Provincial Key Laboratory of Ophthalmology and Visual Science, Guangdong Provincial Clinical Research Center for Ocular Diseases, Guangzhou, Guangdong 510060, China.
Department of Ophthalmology, The First Affiliated Hospital, Sun Yat-Sen University, Guangzhou, Guangdong 510080, China.
J Ophthalmol. 2022 Jul 1;2022:4522974. doi: 10.1155/2022/4522974. eCollection 2022.
This study aimed to describe and analyze the clinical features of 20 eyes of 15 primary vitreoretinal lymphoma (PVRL) patients.
This was a retrospective case series and a review of the literature. Fifteen PVRL patients (20 affected eyes) referred between February 2011 and December 2019 were recruited, and their medical records were retrospectively reviewed.
Among these 15 PVRL patients, seven were men (46.67%), and five had bilateral PVRL (33.33%). The median onset age was 66 ± 9.26 years and six (40%) patients had central nervous system (CNS) involvement, and two of them died of CNS-related complications. The ocular symptoms varied from decreased vision to binocular diplopia. The ocular manifestations were diverse and involved both the anterior and posterior segments, including the vitreous cells, subretinal white-yellow lesions, cotton-wool spots, and ophthalmoplegia. The rate of misdiagnosis and failure to diagnose was 100%, and 30% of them were misdiagnosed as uveitis. We found five cases revealing rare characteristics of this malignancy. Among them, there were two cases with mild hypertensive retinopathy exhibiting cotton-wool spots, one case mimicking age-related macular degeneration (AMD), one case with systemic lupus erythematosus (SLE), and one patient had extraocular muscle involvement. To the best of our knowledge, we reported PVRL exhibiting cotton-wool spots as the main manifestation and coexisting with extraocular myopathy for the first time.
PVRL is a rare intraocular malignancy that commonly masquerades as uveitis. As the clinical signs and symptoms are atypical, ophthalmologists must carefully examine patients to avoid misdiagnosis or a failure to diagnose. Cotton-wool spots and extraocular myopathy might be the dominant initial symptoms in PVRL patients, and AMD should be considered a differential diagnosis of PVRL. SLE patients under immunosuppressive treatment could have spontaneous PVRL.
本研究旨在描述和分析15例原发性玻璃体视网膜淋巴瘤（PVRL）患者20只眼的临床特征。
这是一项回顾性病例系列研究及文献综述。纳入2011年2月至2019年12月间转诊的15例PVRL患者（20只患眼），对其病历进行回顾性分析。
这15例PVRL患者中，男性7例（46.67%），5例为双侧PVRL（33.33%）。发病年龄中位数为66±9.26岁，6例（40%）患者有中枢神经系统（CNS）受累，其中2例死于CNS相关并发症。眼部症状从视力下降到双眼复视不等。眼部表现多样，累及眼前段和后段，包括玻璃体细胞、视网膜下白色 - 黄色病变、棉絮斑和眼肌麻痹。误诊和漏诊率为100%，其中30%被误诊为葡萄膜炎。我们发现5例具有这种恶性肿瘤的罕见特征。其中，2例轻度高血压性视网膜病变伴有棉絮斑，1例类似年龄相关性黄斑变性（AMD），1例患有系统性红斑狼疮（SLE），1例患者有眼外肌受累。据我们所知，我们首次报道了以棉絮斑为主要表现并与眼外肌病共存的PVRL。
PVRL是一种罕见的眼内恶性肿瘤，常伪装成葡萄膜炎。由于临床体征和症状不典型，眼科医生必须仔细检查患者以避免误诊或漏诊。棉絮斑和眼外肌病可能是PVRL患者的主要初始症状，AMD应被视为PVRL的鉴别诊断。接受免疫抑制治疗的SLE患者可能会自发发生PVRL。
