An unusual case report of primary vitreoretinal lymphoma

Abstract

BackgroundPrimary vitreoretinal lymphoma (PVRL) is a rare ocular condition and its diagnosis remains a challenge. The clinical presentation is variable and it can masquerade as chronic intermediate or posterior uveitis. We report an unusual case of primary central nervous system lymphoma (PCNSL) presenting as migrating retinal lesions with unique shapes. The diagnostic challenges are described and the clinical features of intraocular lymphoma are reviewed.Case presentationA 53 year-old gentleman presented with unilateral visual disturbance and a wide area of retinal whitening with sharp borders temporal to the macula, corresponding to hyper-reflective subretinal changes on optical coherence tomography (OCT). The lesion resolved spontaneously after 6 weeks but was replaced by multiple punctate sub-retinal and sub-retinal pigment epithelial lesions. The second eye was involved 4 months later and there were new areas of hyper-reflective changes in both eyes, which migrated nasally within a week, with no evidence of scarring in the previous sites. The diagnosis of diffuse B-cell lymphoma was made on brain biopsy when the patient subsequently presented with acute confusion and magnetic resonance imaging brain scan showed a frontal lesion. Following systemic chemotherapy, the sub-retinal changes resolved with complete restoration of retinal architecture and improvement in visual acuity.ConclusionWe report an unusual case of PVRL presenting as migrating retinal lesions with unique shapes. PVRL is a great imitator and a high index of clinical suspicion is required in unexplained ocular lesions to prevent a delay in diagnosis.

Optical Coherence Tomography Benefits the Diagnosis and Follow-Up of Primary Central Nervous System Lymphoma with Intraocular Involvement

“…Hyperreflective subretinal dots were a sign that arose after the disappearance of lymphoma infiltration and subsequently remained stable in three cases of the VRL group. To our knowledge, only Tan et al 25 reported this phenomenon. These persistent hyperreflective dots may be the debris of lymphoma cells, degenerated RPE cells, or degenerated photoreceptor outer segments that were non-absorbable by the RPE layer.…”

“…Another possible explanation is that vitreoretinal lymphoma is able to regress spontaneously, although only several cases have been reported. 25–27 …”

Rapid, Spontaneous Resolution of Prominent Subretinal Infiltrate in Vitreoretinal Lymphoma

“…16 Tan and colleagues described a patient with a subretinal infiltrate that spontaneously resolved after 6 weeks who later underwent a brain biopsy showing DLBCL. 17 Pakdel and colleagues reported 2 cases of VRL with subretinal infiltrates that regressed soon after diagnostic PPV. They hypothesized that this could have been from PPV-induced T-cell inflammation, which caused suppression of neoplastic B lymphocytes.…”

Research on the Damage of the Central Nervous System Lymphoma to the Nervous System

“…Visual disturbances are common in PCNSL. One 53-year-old man presented with wide retinal whitening who's PCNSL presented as migrating retinal lesions [75] . Finally, in a case of PCNSL limited to the intramedullary spinal cord, the patient had tremendous lower back pain and weakness in his lower extremities [76] .…”