Xu Lucy T, Huang Ye, Liao Albert, Anthony Casey L, Voloschin Alfredo, Yeh Steven
Retina Group of Washington, Washington, DC, USA.
Truhlsen Eye Institute, University of Nebraska Medical Center, Omaha, NE, USA.
Int J Retina Vitreous. 2022 Aug 26;8(1):58. doi: 10.1186/s40942-022-00405-0.
Primary vitreoretinal lymphoma (PVRL) is an aggressive lymphoma that may present with protean features and represents a diagnostic challenge. Given that patients with PVRL are at high risk of CNS involvement with a high mortality and morbidity rate, prompt diagnosis is crucial to initiate treatment early in the disease course. A multimodality imaging approach including fundus photography, fundus autofluorescence (FAF), optical coherence tomography (OCT), fluorescein and indocyanine angiography, and electroretinography (ERG) can provide information to establish a diagnosis and provide objective measures for management. We review key findings seen via these imaging modalities in patients with PVRL.
Fundus photography can highlight commonly seen patterns of PVRL including vitritis, subretinal disease, retinal pigment epithelial (RPE) abnormalities, optic nerve edema, retinal detachment, and less typical retinitis-like lesions. FAF can identify characteristic patterns of hyper- and hypoautofluorescent signal abnormalities in the macula. Spectral-domain OCT will demonstrate vitreous cells, RPE nodularity, and hyperreflectivity of the outer retina. The presence of a hyper-reflective band in the subretinal space and infiltrates between the RPE and Bruch's membrane can assist in distinguishing PVRL from choroidal lymphoma. Vertical hyperreflective columns (VHRLs) are another pertinent finding that may represent microinfiltrates of the tumor. OCT has proven to be a particularly useful modality in assessing the progress of treatment in PVRL. Fluorescein angiography can show RPE changes, which include granularity, late staining at the RPE level, and blockage. Indocyanine green angiography (ICGA) primarily shows hypocyanescence, which corresponds to PVRL lesions on fundus photography and may occur secondary to loss of RPE and choriocapillaris.
While PVRL remains a challenging disease to diagnose and follow, the use of a multimodality imaging approach may assist in establishing a diagnosis. Because of the anatomic spaces PVRL may affect, fundus photography, OCT, FAF, angiography, and ERG can identify key characteristics of the disease, differentiate PVRL from other diseases, and provide baseline information for targeted systemic and local therapies. Further assessment of anatomic and functional targets will aid our clinical application of multimodal imaging in the management of PVRL.
原发性玻璃体视网膜淋巴瘤（PVRL）是一种侵袭性淋巴瘤，可能具有多种表现形式，构成诊断挑战。鉴于PVRL患者发生中枢神经系统受累的风险高，死亡率和发病率也高，早期诊断对于在疾病进程中尽早开始治疗至关重要。包括眼底照相、眼底自发荧光（FAF）、光学相干断层扫描（OCT）、荧光素和吲哚菁绿血管造影以及视网膜电图（ERG）在内的多模态成像方法可以提供信息以确立诊断，并为治疗提供客观指标。我们回顾了PVRL患者通过这些成像方式所观察到的关键发现。
眼底照相可突出显示PVRL常见的表现形式，包括玻璃体炎、视网膜下病变、视网膜色素上皮（RPE）异常、视神经水肿、视网膜脱离以及不太典型的视网膜炎样病变。FAF可识别黄斑区自发荧光信号异常的特征性模式。谱域OCT将显示玻璃体细胞、RPE结节以及视网膜外层的高反射性。视网膜下间隙中高反射带的存在以及RPE与布鲁赫膜之间的浸润有助于将PVRL与脉络膜淋巴瘤区分开来。垂直高反射柱（VHRLs）是另一个相关发现，可能代表肿瘤的微浸润。OCT已被证明是评估PVRL治疗进展的一种特别有用的方式。荧光素血管造影可显示RPE改变，包括颗粒状、RPE水平的晚期染色以及遮蔽现象。吲哚菁绿血管造影（ICGA）主要显示低荧光，这与眼底照相上的PVRL病变相对应，可能继发于RPE和脉络膜毛细血管的丧失。
虽然PVRL仍然是一种诊断和随访都具有挑战性的疾病，但使用多模态成像方法可能有助于确立诊断。由于PVRL可能影响的解剖间隙，眼底照相、OCT、FAF、血管造影和ERG可以识别该疾病的关键特征，将PVRL与其他疾病区分开来，并为有针对性的全身和局部治疗提供基线信息。对解剖和功能靶点的进一步评估将有助于我们在PVRL管理中临床应用多模态成像。
