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.
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.
Authors: Gregory S Vosganian; Saskia Boisot; Kathrin I Hartmann; William R Freeman; Robert W Sharpe; Prabhakar Tripuraneni; Alan Saven Journal: J Neurooncol Date: 2011-05-31 Impact factor: 4.130