Intraocular lymphoma

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Fahimeh Shojaei, M.D., Maria Fernanda Villarreal, M.D. [2]

Synonyms and Keywords: PIOL; Ocular Lymphoma; Primary central nervous system ocular lymphoma

Overview

Intraocular lymphoma is a rare subtype of primary central nervous system lymphoma. Intraocular lymphoma may affect the eye secondarily from a metastasis from a non-ocular tumor or may arise within the eye primarily. According to the World Health Organization (WHO) classification, intraocular lymphomas are most commonly a diffuse large B-cell immunohistologic subtype of non-Hodgkin's lymphoma. The most common symptoms of intraocular lymphoma is blurred or decreased vision. In the majority of patients, central nervous system involvement, only accounts for 20% of primary central nervous system lymphomas. The median age at diagnosis among patients with intraocular lymphoma is between 50-60 years. The pathogenesis of intraocular lymphoma is characterized by the affection of the sub-retinal pigment epithelium, which is normally involved in the light absorption. On fluorescein angiography, findings may reveal "leopard spot" patterns due to sub-retinal pigment epithelium infiltrates that stain early and progressively or mottling of the sub-retinal pigment epithelium due to hyper- and hypo-fluorescent window defects. The initial therapy for patients with intraocular lymphoma is corticosteroids.

Historical Perspective

  • Intraocular lymphoma was first discovered by Samuel Wilks, a British physician, in 1856.[1]

Classification

Intraocular lymphoma may be classified according to its location into 2 subtypes:[2]

  • Retinal
    • Retinal
    • Vitreoretinal
    • Vitreal
  • Uveal
    • Iridal
    • Ciliary
    • Choroidal



 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Intraocular lymphoma
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Retinal
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Uveal
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Retinal
 
 
 
Vitreoretinal
 
 
 
Vitreal
 
 
 
Iridal
 
 
 
Ciliary
 
 
 
Choroidal
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Primary
 
Secondary


Pathophysiology

  • Intraocular lymphoma arises from B cell, which are normally involved in immune system.
  • Intraocular lymphoma can be either primary or secondary central nervous system lymphoma (oculocerebral lymphoma) that mainly affects the optic nerve and the eye.[3]
  • The pathogenesis of intraocular lymphoma is characterized by the affection of the sub-retinal pigment epithelium, which is normally involved in the light absorption.[4]
  • Genes associated with the development of intraocular lymphoma, include:[3]
  • On microscopic histopathological analysis, characteristic findings of intraocular lymphoma, include:[3]
  • Marginal zone (52%)
  • Follicular (23%)
  • Atypical lymphocytes (gold standard)
  • Elevated interleukin IL-10 According to the World Health Organization (WHO) classification, intraocular lymphomas are most commonly a diffuse large B-cell immunohistologic subtype of non-Hodgkin's lymphoma.[4]

Causes

  • There are no established causes of intraocular lymphoma.

Differentiating Intraocular Lymphoma from Other Diseases

  • Intraocular lymphoma must be differentiated from other diseases that cause chronic loss of vision, or headaches, such as:[4][5]

Epidemiology and Demographics

Prevalence

  • The prevalence of intraocular lymphoma remains unknown.[4][6]

Age

  • Intraocular lymphoma is more commonly observed among patients aged 50 to 60 years old.

Gender

  • Males are more commonly affected with intraocular lymphoma than females.

Race

  • There is no racial predilection for intraocular lymphoma.

Risk Factors

  • Common risk factors in the development of intraocular lymphoma, include:[7][8]
  • Toxoplasma gondii infection
  • EBV infection
  • HHV8 infection

Natural History, Complications and Prognosis

  • The majority of patients with intraocular lymphoma are symptomatic at the time of diagnosis.
  • Early clinical features include symptoms of a non-resolving uveitis, and blurred vision.[4]
  • If left untreated, patients with intraocular lymphoma may progress to develop complete vision loss.
  • Common complications of intraocular lymphoma, include:[3]
  • Blindness
  • Radiation-induced retinopathy
  • Neovascular glaucoma
  • Prognosis is generally good, and the 5-year survival for patients with intraocular lymphoma is between 65-80%.[3]

Diagnosis

History and Symptoms

  • Common symptoms of intraocular lymphoma include:[4][9]

Physical Examination

  • Patients with intraocular lymphoma usually appear pale or malnourished.[4]
  • Physical examination may be remarkable for:
  • Decreased visual acuity (most common)
  • Irregular pupil
  • Increased lacrimation
  • Eye redness
  • Increased intraocular pressure

Laboratory Findings

  • There are no specific laboratory findings associated with intraocular lymphoma.

Imaging Findings

  • Magnetic resonance image (MRI) is the imaging modality of choice for patients with intraocular lymphoma.[4]
  • On MRI, characteristic findings of intraocular lymphoma, include:[3]
  • Fat suppressed T2 and post-contrast T1 weighted images, with thin slice thickness and a reduced FOV is ideal, and should include the cavernous sinuses.
  • T1: isointense to muscle 8
  • T2: hyperintense to muscle, hypointense to fat
  • T1 C+ (GAD): enhancement present but variable

Treatment

Medical Therapy

  • The initial therapy for intraocular lymphoma is corticosteroids.[4]
  • Other medical therapies for intraocular lymphoma, may include:[4]
  • Methotrexate
  • Intravitreal rituximab
  • Localized external beam radiation therapy (EBRT)

Surgery

  • Surgery is the mainstay of therapy for intraocular lymphoma.

Primary Prevention

  • There are no primary preventive measures available for intraocular lymphoma.[4]
  • Once diagnosed and successfully treated, patients with intraocular lymphoma are followed-up periodically every 3, 6, or 12 months.
  • The average relapse rate among patients with intraocular lymphoma is 2 years.[4]

Secondary Prevention

References

  1. Ahmed S, Shahid RK, Sison CP, Fuchs A, Mehrotra B. Orbital lymphomas: a clinicopathologic study of a rare disease. Am J Med Sci. 2006 Feb. 331(2):79-83.
  2. Coupland, Sarah E; Damato, Bertil (2008). "Understanding intraocular lymphomas". Clinical & Experimental Ophthalmology. 36 (6): 564–578. doi:10.1111/j.1442-9071.2008.01843.x. ISSN 1442-6404.
  3. 3.0 3.1 3.2 3.3 3.4 3.5 Intraocular lymphoma. Wikipedia. https://en.wikipedia.org/wiki/Intraocular_lymphoma
  4. 4.00 4.01 4.02 4.03 4.04 4.05 4.06 4.07 4.08 4.09 4.10 4.11 Hanson JA, Alexandru D and Bota DA: The evaluation and treatment of primary intraocular lymphoma. journal of Cancer Therapeutics and Research 2013, 2:15 http://dx.doi.org/10.7243/2049-7962-2-15
  5. Sen, H. Nida; Bodaghi, Bahram; Hoang, Phuc Le; Nussenblatt, Robert (2009). "Primary Intraocular Lymphoma: Diagnosis and Differential Diagnosis". Ocular Immunology and Inflammation. 17 (3): 133–141. doi:10.1080/09273940903108544. ISSN 0927-3948.
  6. Mochizuki, Manabu; Singh, Arun D. (2009). "Epidemiology and Clinical Features of Intraocular Lymphoma". Ocular Immunology and Inflammation. 17 (2): 69–72. doi:10.1080/09273940902957305. ISSN 0927-3948.
  7. Shen, De Fen; Herbort, Carl P; Tuaillon, Nadine; Buggage, Ronald R; Egwuagu, Charles E; Chan, Chi-Chao (2001). "Detection of Toxoplasma Gondii DNA in Primary Intraocular B-Cell Lymphoma". Modern Pathology. 14 (10): 995–999. doi:10.1038/modpathol.3880424. ISSN 0893-3952.
  8. Chan CC (2003). "Molecular pathology of primary intraocular lymphoma". Trans Am Ophthalmol Soc. 101: 275–92. PMC 1358994. PMID 14971583.
  9. Coupland, Sarah E; Damato, Bertil (2008). "Understanding intraocular lymphomas". Clinical & Experimental Ophthalmology. 36 (6): 564–578. doi:10.1111/j.1442-9071.2008.01843.x. ISSN 1442-6404.

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