Intraocular Lymphoma

Abstract

Background Primary ocular lymphoma is a distinct subtype of intraocular lymphoma. Its clinical presentation can mimic benign conditions. Diagnosis is often based on obtaining an intraocular biopsy. Optimal management is not yet realized. Methods This report combines the experience of the author with a review of the current literature pertaining to intraocular lymphoma. Results Primary ocular lymphoma, a subtype of primary central nervous system (CNS) lymphoma, has a variable clinical course and frequently mimics benign inflammatory disease. Even when suspected, diagnosis can be elusive. Chemoradiation is the most effective treatment, but significant ocular and cerebral morbidity is associated with its use. Novel treatment regimens may reduce or eliminate side effects while preserving life, vision, and CNS function. Conclusions Primary CNS lymphoma with ocular involvement should be considered in patients with refractory uveitis, yellow-white choroidal masses, and CNS lymphoma. Aggressiveness in making the diagnosis should be tempered by the potential complications of the diagnostic process and advanced by the life-threatening nature of the disease. Treatment should attempt to maximize efficacy while incorporating considerations such as extent of disease and the patient's age, health, and mental state.

Primary Intraocular Lymphoma: A Review

“…Vitreous samples typically contain few lymphocytes, and a number of them may be inflammatory, not neoplastic (Bardenstein, 1998). In many cases, the paucity of lymphoma cells is due to previous treatment for presumed uveitis with corticosteroids, which is cytolytic to them (Whitcup et al, 1993).…”

“…This type of lymphoma is estimated to represent 1% of non-Hodgkin's lymphomas, 1% of intracranial tumors, and less than 1% of intraocular tumors (Bardenstein, 1998). Several reports have estimated that 15-25% of patients with PCNSL will have ocular involvement.…”

“…In many cases, the paucity of lymphoma cells is due to previous treatment for presumed uveitis with corticosteroids, which is cytolytic to them (Whitcup et al, 1993). Multiple biopsy attempts may be required before the diagnosis of IOL is made (Bardenstein, 1998).…”

Primary intraocular lymphoma: a review

“…PIOL is estimated to represent 1% of NHLs, 1% of intracranial tumors and less than 1% of intraocular tumors [4]. Approximately 1,000 people per year are diagnosed with PCNSL in the United States and of those cases 15-25% will have ocular involvement at the time of diagnosis [2].…”

“…Bilateral vitrectomies were performed revealing a population of intermediate to large cells with a high nuclear to cytoplasmic ratios, irregular nuclear contours and variably prominent nucleoli (Figs. 3,4). Flow cytometry revealed lambda light chain restricted monoclonal B-cells (Fig.…”