Primary Intraocular Diffuse Large B-cell Lymphoma: Diagnostic Difficulties in Deep Retinal Infiltrations with Vitritis

Abstract

Purpose Primary intraocular lymphoma (PIOL) is a rare malignancy with an aggressive clinical course. It is usually considered as a subset of primary central nervous system lymphoma. Differential diagnosis should include infectious and non-infectious aetiologies, particularly the common masqueraders sarcoidosis, tuberculosis, viral retinitis and syphilis. Patient The article presents a case of bilateral vitreoretinal lymphoma manifesting as uveitis and vitritis resistant to corticosteroid therapy. The final diagnosis was based on a retinal biopsy. Results The patient was successfully treated with systemic and local therapy. Long-term complete remission (CR) was reached. The relapse of diffuse large B-cell lymphoma was revealed in the frontal left lobe after 48 months of CR duration. Conclusion The diagnosis of PIOL is always very difficult. Cooperation of pathologists, ophthalmologists and hematologists is required for a quick and accurate diagnosis. Local and systemic treatment is needed to achieve CR, but the relapse rate remains very high.

Rapidly Progressive Bilateral Vitreoretinal Lymphoma

“…Primary vitreoretinal lymphoma (PVRL) is an ocular malignancy that is defined as a subset of primary central nervous system lymphoma (PCNSL) in which lymphoma cells are initially present only in the eyes without evidence of disease in the brain, spinal cord, leptomeninges, or cerebrospinal fluid [3][4][5][6][7]. PVRL follows a particularly aggressive clinical course and can be challenging to diagnose due to its ability to masquerade as noninfectious or infectious uveitis, or other neoplasms such as metastatic cancers [1]. The presentation of PVRL can be diverse and non-specific, with vitritis present in 66% of cases and prototypical cream-colored sub-retinal yellow infiltrates being reported in only 41% of cases [8].…”

“…Primary vitreoretinal lymphoma (PVRL) is often considered a subset of primary central nervous system (CNS) lymphoma that follows a particularly aggressive clinical course and can be challenging to diagnose due to its ability to masquerade as other more common causes of uveitis [ 1 ]. The differential diagnosis includes infectious etiologies such as viral retinitis, Bartonella, tick-borne infections, tuberculosis, and syphilis, and non-infectious etiologies such as sarcoidosis, acute posterior multifocal placoid pigment epitheliopathy (APMPPE), and Behcet’s disease.…”

“…The differential diagnosis includes infectious etiologies such as viral retinitis, Bartonella, tick-borne infections, tuberculosis, and syphilis, and non-infectious etiologies such as sarcoidosis, acute posterior multifocal placoid pigment epitheliopathy (APMPPE), and Behcet’s disease. High index of clinical suspicion for vitreoretinal lymphoma, with careful consideration of the patient's immune status and the characteristics of intraocular inflammation, requires prompt biopsy for quick and accurate diagnosis to prevent rapid intraocular progression and further CNS involvement [ 1 , 2 ]. We herein report a case of large B-cell vitreoretinal lymphoma that initially presented with unilateral involvement and within one month had undergone rapid progression in the affected eye.…”