Abstract
Vitreoretinal lymphoma is an intraocular lymphoma that mimics intermediate, posterior, or panuveitis in older patients who are not responsive to steroid therapy; typical subtype is diffuse, large B-cell, non-Hodgkin’s. Primary intraocular lymphoma (PIOL) denotes absence of CNS involvement.
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Keywords
Overview
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Definition
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Intraocular lymphoma mimicking intermediate, posterior, or panuveitis in older patients not responsive to steroid therapy; typical subtype is diffuse, large B-cell, non-Hodgkin’s
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Primary intraocular lymphoma (PIOL) denotes absence of central nervous system (CNS) involvement
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Symptoms
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Blurry vision
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Floaters
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Scotoma
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Frank inflammatory symptoms such as redness, pain, and photophobia are rare unless there is significant anterior segment infiltration
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Laterality
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80% bilateral
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Course
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Insidious and progressive
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Age of onset
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50–60 years (range 15–85)
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Gender/race
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Slight female and Caucasian predilection
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Systemic association: CNS lymphoma
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20% of CNS lymphoma patients have ocular disease at the time of diagnosis; however
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If intraocular lymphoma is diagnosed first, 60–80% develop CNS disease after 2–3 years
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Overall, intraocular-CNS lymphoma makes up only 1% of all non-Hodgkin’s lymphoma in immunocompetent patients (200 cases diagnosed a year in the United States)
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Higher incidence of intraocular-CNS lymphoma in acquired immunodeficiency syndrome (AIDS), post-organ transplant, and congenital immunodeficiencies
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Exam: Ocular
Anterior Segment
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No or very mild external sign of inflammation
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Conjunctival hyperemia
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Keratic precipitates
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Mild Anterior Chamber reaction
Posterior Segment
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Large clumps or sheets of cells in vitreous
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Mild to very dense vitritis
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Multifocal, large, yellow sub-RPE or subretinal infiltrates
Exam: Systemic
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Headache
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Personality change
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Alertness alteration
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Memory loss
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Nausea/vomiting
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Gait imbalance
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Weakness
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Seizure
Imaging
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OCT: Sub-RPE hyper-reflective material; no CME
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FA: RPE granularity and possible blockage corresponding to sub-RPE infiltrates, but no vasculitis, papillitis, or macular leakage that would be expected in inflammatory syndromes
Laboratory and Radiographic Testing
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Gold standard for ocular diagnosis is either
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Vitreous biopsy or sub-RPE aspirate via pars plana vitrectomy; not uncommon to need repeat biopsy if first one negative; ALL following analyses are recommended to have best yield
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Undiluted sample for cytopathology
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Diluted sample for molecular genetics to look for monoclonality
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IgH gene rearrangement: B-cell lymphoma
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T-cell receptor gene rearrangement: T-cell lymphoma
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Diluted sample for interleukin (IL)-6 and -10 levels
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High IL-10/6 consistent with lymphoma
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Chorioretinal biopsy
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Used when there is obvious, focal chorioretinal lesion but little vitreous cellularity
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Significantly higher ocular morbidity than vitreous biopsy alone (retinal detachment, vitreous hemorrhage, proliferative vitreoretinopathy, etc.)
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MRI brain to assess extent of any CNS involvement
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Deep brain structures, especially front lobe, are more often involved than cerebral cortex compared to other brain tumors (thus change in personality and alertness).
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Supratentorial and multicentric in half the cases; characteristically dense and diffuse enhancement with distinct borders.
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Positron emission tomography (PET) (may not detect Primary intraocular lymphoma [PIOL])
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Lumbar puncture: Required in all patients suspected of CNS lymphoma
Differential Diagnosis
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Sarcoidosis
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Tuberculosis
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White dot syndromes, especially Birdshot
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White dot syndrome in an older patient ➔ think lymphoma
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Viral retinitis
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Lymphoid hyperplasia of the uvea (usually unilateral and responds well to steroids)
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Amelanotic uveal melanoma
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Uveal metastasis
Treatment
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Local therapies
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Intravitreal methotrexate (MTX) 400 mcg/0.1 cc or rituximab (RTX) 1 mg/0.1 cc weekly for 6 weeks, extended as needed if cellularity or lesion persists; repeat cycles as needed
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If subretinal lymphoma is threatening macula and there is little suspicion for infectious uveitis, then empiric intravitreal MTX may be appropriate before biopsy result is available
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External beam radiation
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Given the high rate of eventual CNS involvement, we recommend systemic chemotherapy as prophylaxis even if there is no CNS involvement at the time of diagnosis
Referral/Co-management
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Neuro-oncology
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Radiation oncology
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Neurology
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Small, K.W. (2021). Vitreoretinal Lymphoma. In: Foster, C.S., Anesi, S.D., Chang, P.Y. (eds) Uveitis. Springer, Cham. https://doi.org/10.1007/978-3-030-52974-1_62
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DOI: https://doi.org/10.1007/978-3-030-52974-1_62
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