
Contents
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Introduction and definition Introduction and definition
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Epidemiology and aetiology Epidemiology and aetiology
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Histopathology and molecular pathogenesis Histopathology and molecular pathogenesis
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Pathological features Pathological features
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Pathogenesis Pathogenesis
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Pathological differential diagnosis Pathological differential diagnosis
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T-cell lymphomas T-cell lymphomas
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Secondary vitreoretinal lymphomas Secondary vitreoretinal lymphomas
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Uveal lymphoma Uveal lymphoma
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Diagnosis Diagnosis
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Symptoms and signs Symptoms and signs
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Ocular imaging Ocular imaging
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Brain and body imaging Brain and body imaging
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Diagnostic approaches Diagnostic approaches
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Management Management
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Overview of treatment Overview of treatment
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Surgery and intraocular chemotherapy Surgery and intraocular chemotherapy
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Radiotherapy Radiotherapy
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Systemic pharmacotherapy Systemic pharmacotherapy
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Chemotherapy Chemotherapy
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Emerging therapies Emerging therapies
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High-dose chemotherapy and autologous stem cell transplantation High-dose chemotherapy and autologous stem cell transplantation
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Bruton’s tyrosine kinase inhibitors Bruton’s tyrosine kinase inhibitors
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Immunomodulatory drugs Immunomodulatory drugs
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Survivorship, quality of life, and palliative care Survivorship, quality of life, and palliative care
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Surveillance Surveillance
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Guidelines Guidelines
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References References
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Cite
Abstract
Primary vitreoretinal lymphoma (PVRL) represents <1% of all intraocular tumours. The diagnosis of PVRL is challenging and clinical symptoms often mimic chronic uveitis. Multimodality ocular imaging is useful when there is a suspicion of PVRL, but pathological confirmation is essential. Ninety-five per cent of PVRL cases are diffuse large B-cell lymphomas. Additional morphological analyses performed on the vitreous and aqueous humour have improved the diagnostic yield. Local radiotherapy or intravitreal injection of steroid and/or low doses of methotrexate results in ocular remission in >80% of cases, but there is a high rate of ocular and/or central nervous system (CNS) relapse within 2 years. High-dose intravenous methotrexate, with or without local treatment, is being investigated in PVRL, with the dual aims of obtaining local disease control and preventing CNS relapse. This chapter covers the epidemiology, pathogenesis, diagnosis, and treatment options for PVRL.
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