Multimodal diagnostic imaging in primary vitreoretinal lymphoma

Abstract

Background: 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.

Observations: 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.

Conclusion: 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.

Keywords: Electroretinography; Fluorescein angiography; Fundus autofluorescence; Fundus photography; Indocyanine green angiography; Multimodal imaging; Optical coherence tomography; Primary vitreoretinal lymphoma.

Fig. 1

Clinical imaging features of a patient with a diagnosis of PVRL and macular degeneration. A Color fundus photograph of the right eye showing vitreous opacity and multiple chorioretinal lesions with variable pigmentation B Fluorescein angiography showing staining of chorioretinal lesions and retinal pigment epithelial (RPE) changes. C Fundus autofluorescence of the right eye showing corresponding areas of hypoautofluorescence. D OCT showing RPE elevation and overlying cystoid edema

Fig. 2

Clinical imaging features of a patient with primary central nervous system lymphoma and recurrent PVRL. A Color fundus photograph of the left eye showing multiple hypopigmented lesions. B Fundus autofluorescence of the left eye showing corresponding hyperautofluorescent spots within the posterior pole and mid-periphery. C OCT showing nodular lesions at the level of the RPE and outer retinal/ellipsoid zone irregularity (yellow arrows)

Fig. 3

Clinical and imaging features of a patient with a diagnosis of PVRL. A Color fundus photograph of the left eye showing discrete yellow chorioretinal lesions (black arrow). B Fundus autofluorescence showing small, hypoautofluorescent spots (small white arrows) consistent with PVRL. C OCT showing hyperreflective RPE clumping and irregularity (white arrow with black outline) consistent with PVRL