Szekeres Denes, Parker Jonathan, Risch Evan, Vibhute Prasanna, Bathla Girish, Agarwal Shweta, Agarwal Amit, Soni Neetu
From the University of Rochester (D.S.), School of Medicine and Dentistry, Rochester, New York.
Eastern Virginia Medical School (J.P.), Norfolk, Virginia.
AJNR Am J Neuroradiol. 2025 Apr 2;46(4):652-658. doi: 10.3174/ajnr.A8517.
Primary intraocular lymphoma (PIOL) is a rare form of primary central nervous system lymphoma that poses diagnostic challenges because of its nonspecific clinical features and complex imaging characteristics. This paper presents a focus case and 2 companion cases, highlighting the complexities in identifying and treating PIOL. In the focus case, a 66-year-old man experienced gradual painless vision loss with choroidal thickening on funduscopic examination and subsequent follow-up MRI. Transvitreal biopsy confirmed PIOL, and the patient was treated with intravitreal steroids and systemic rituximab without recurrence. Companion case 1 involved a 66-year-old woman with vision changes and choroidal thickening with episcleral extension on MRI suggestive of intraocular lymphoma and ultimately treated with radiation with the presumed diagnosis of PIOL. In the companion case 2, a 63-year-old man with ocular symptoms was diagnosed with chronic lymphocytic leukemia along with vitreoretinal Richter transformation. Enucleation was performed because of a lack of visual potential and failure of chemotherapy, which confirmed PIOL. Distinguishing PIOL from other ocular conditions is crucial, given its potential for CNS involvement. Imaging plays a vital role in corroborating clinical findings. While cytology remains the standard for diagnosis, supplementary tests, including cytokine analysis, immunohistochemistry, and flow cytometry, provide additional insights. PIOL treatment strategies are tailored to disease extent, ranging from locoregional chemotherapy to invasive enucleation. CNS involvement carries a poor prognosis and must evaluated and surveilled with MRI. In conclusion, this case series reviews the clinical and radiologic features of PIOL, emphasizing the significance of diagnostic imaging in determining disease extent and guiding treatments.
原发性眼内淋巴瘤(PIOL)是原发性中枢神经系统淋巴瘤的一种罕见形式,因其非特异性临床特征和复杂的影像学特征而带来诊断挑战。本文介绍了1例重点病例和2例对照病例,突出了PIOL诊断和治疗中的复杂性。在重点病例中,一名66岁男性逐渐出现无痛性视力丧失,眼底检查及后续的MRI检查显示脉络膜增厚。经玻璃体活检确诊为PIOL,患者接受了玻璃体内注射类固醇和全身使用利妥昔单抗治疗,未复发。对照病例1为一名66岁女性,有视力变化,MRI显示脉络膜增厚并伴有巩膜外扩展,提示眼内淋巴瘤,最终在推定诊断为PIOL的情况下接受了放疗。在对照病例2中,一名63岁男性有眼部症状,被诊断为慢性淋巴细胞白血病合并玻璃体视网膜里氏转化。由于视力恢复无望且化疗失败,遂行眼球摘除术,术后确诊为PIOL。鉴于PIOL可能累及中枢神经系统,将其与其他眼部疾病区分开来至关重要。影像学在佐证临床发现方面起着至关重要的作用。虽然细胞学检查仍是诊断的金标准,但包括细胞因子分析、免疫组织化学和流式细胞术在内的补充检查可提供更多信息。PIOL的治疗策略根据疾病范围进行调整,从局部化疗到侵入性眼球摘除术不等。中枢神经系统受累预后较差,必须通过MRI进行评估和监测。总之,本病例系列回顾了PIOL的临床和放射学特征,强调了诊断性影像学在确定疾病范围和指导治疗方面的重要性。