Secondary vitreoretinal lymphoma with spontaneous regression

Abstract

Purpose: To report a patient with vitreoretinal lymphoma (VRL) secondary to systemic diffuse large B-cell lymphoma, who had two episodes of spontaneous regression.

Observations: An 80-year-old Nicaraguan male with a history of treated systemic diffuse large B-cell lymphoma presented with decreased vision in his right eye over one year. The patient was found to have subretinal lesions and moderate vitreous opacities in his right eye. Cytological analysis of vitreous confirmed B-cell lymphoma. Following his systemic work-up, spontaneous clinical improvement was noted. There were no vitreoretinal or systemic lymphoma recurrences during one year of follow-up until the patient had new onset decreased vision in the left eye. He was presumed to have a recurrence of VRL supported by optical coherence tomography findings. Repeat systemic workup was negative for reoccurrence and the ocular lesions resolved spontaneously over 4 weeks.

Conclusions: Spontaneous regression of intraocular lymphoma can rarely occur. Multimodal imaging has an essential role in diagnosing and monitoring recurrence of this disease.

Keywords: Large B-cell lymphoma; Multimodal imaging; Recurrence; Spontaneous regression; Vitreoretinal lymphoma.

Fig. 1

Multimodal imaging of the right eye at initial presentation. Color fundus photograph showed diffuse subretinal and retinal infiltrates, affecting predominantly the optic disc and the peripapillary area (A) with higher magnification (B) OCT macula showed diffuse retinal thickening with RPE irregularity as well as hyperreflective subretinal (asterisk) and sub-RPE deposits (arrow head) (C) Fundus autofluorescence showed area of hypo-autofluorescence corresponding to infiltrates surrounded by mottled pattern of hyper-and hypo-autofluorescence (D) Fluorescein angiography (FA) of the right eye showed leakage of the optic disc and peripapillary areas, as well as staining of lymphomatous lesions on early (E) and late frames (F). (For interpretation of the references to colour in this figure legend, the reader is referred to the Web version of this article.)

Fig. 2

Multimodal imaging of the right eye at 2 months after initial presentation. Color fundus photograph showed spontaneous improvement of intraretinal and subretinal infiltrates (A) Fundus autofluorescence revealed decreased hypo-autofluorescence at the optic disc and the peripapillary area with a mottled pattern of hyper-and hypo-autofluorescence (B) OCT macula showed diffuse loss of outer retina layers, subretinal fibrosis, and an epiretinal membrane (C). (For interpretation of the references to colour in this figure legend, the reader is referred to the Web version of this article.)

Fig. 3

Sequential OCT of the inferior macula. At initial presentation, OCT showed normal foveal contour without intraretinal lesion (A). At 1 year after initial presentation, disruption of ellipsoid zone (EZ) and a vertical hyperreflective lesion (VHRL) were noted (B). At 1 week after (B), spontaneous reconstitution of EZ was demonstrated and VHRL decreased in size and intensity (C). At 1 month follow up, EZ had fully reconstituted and VHRL completely resolved (D).