Multimodal Imaging of Spontaneously Shifting Primary Vitreoretinal Lymphoma

Abstract

Purpose: To correlate spectral domain optical coherence tomography (SD-OCT) and photographic imaging before and after spontaneous regression of primary vitreoretinal lymphoma (PVRL) lesions.

Procedures: We report the case of a 60-year-old female.

Results: The patient presented with bilateral creamy deposits under the retina and retinal pigment epithelium (RPE), and lesions were visible along Bruch's membrane with SD-OCT and suspicious for PVRL. Systemic workup revealed nonspecific areas of enhancement on neuroimaging. The patient was largely asymptomatic and the decision was made to observe her. Three months later, a new lesion pattern had developed. The color fundus photographs and SD-OCT demonstrated spontaneous regression of the largest sub-RPE lesion, leaving areas of RPE atrophy, while a new larger sub-RPE lesion had formed in the other eye. Vitreous biopsy showed lymphocytes and no malignant cells, while sub-RPE biopsy of the newly formed lesion revealed highly atypical cells positive for CD19 and CD20.

Conclusions: Multimodal imaging documents that PVRL lesion regression and early RPE changes can develop within a 3-month period. Immune control is an important factor in lesion regression in the eye.

Keywords: Immune surveillance; Primary vitreoretinal lymphoma; Spectral domain optical coherence tomography; Spontaneous regression.

Fig. 1

Montage fundus photographs of the OD and OS at presentation (a, c). Note the creamy sub-RPE infiltrates in the macula, temporally, and superiorly in the OD (a) and the coalesced, fluffy sub-RPE infiltrates inferior to the inferior arcade and the discrete infiltrates superonasally in the OS (c). Three months later, many of these lesions resolved without any treatment (b OD, d OS), while a new sub-RPE lesion appeared at 10 o'clock in the OD (b) and temporal to the macula in the OS (d). SD-OCT in the area of the inferior lesion on presentation (e) showed RPE elevation secondary to prominent sub-RPE material accumulation along Bruch's membrane (arrows) and some small subretinal material accumulation, with blocked visualization of much of the choriocapillaris in the OS. Three months later, the area where the lesion regressed showed RPE atrophy and irregularity (arrows) and improved visualization of the choriocapillaris on SD-OCT (f).

Fig. 2

a Fundus autofluorescence photograph of the OS at initial presentation showed a mixed pattern of increased and decreased autofluorescence in the areas of sub-RPE infiltrates. Laminar phase of the fluorescein angiogram at the same visit showed blockage of the choroidal circulation (b) while, in the late phase, there was mild hyperfluorescence of the subretinal lesions with fluffy appearing borders in a leopard pattern (c). d Brain MRI after the first visit was significant for some T2/flair hyperintensities in the white matter.