Intraocular lymphoma

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

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

Overview

Intraocular lymphoma (also known as "PIOL") 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.[1]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.[2]

Historical Perspective

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

Classification

  • 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.[2]
  • Primary central nervous system lymphoma may be classified into 5 subtypes:[2]
  • Primary cerebral lymphoma
  • Primary leptomeningeal lymphoma
  • Primary intraocular lymphoma
  • Primary spinal lymphoma
  • Neurolymphomatosis

Pathophysiology

  • Intraocular lymphoma is characterized as a secondary central nervous system 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.[2]
  • Genes associated with the development of intraocular lymphoma, include:[3]
  • On gross pathology, characteristic findings of intraocular lymphoma, include:
  • No remarkable findings
  • On microscopic histopathological analysis, characteristic findings of intraocular lymphoma, include:[3]
  • Marginal zone (52%)
  • Follicular (23%)
  • Atypical lymphocytes (gold standard)
  • Elevated interleukin IL-10

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:[2]
  • Ocular metastasis (most common)
  • Choroidal hemangioma
  • Vitrous lymphoma
  • Retrolental fibroplasia

Epidemiology and Demographics

  • The prevalence of intraocular lymphoma remains unknown.[2]

Age

  • Intraocular lymphoma is more commonly observed among patients aged 50 to 60 years old.[2]
  • Intraocular lymphoma is more commonly observed among middle aged adults.

Gender

  • Males are more commonly affected with intraocular lymphoma than females.[3]

Race

  • There is no racial predilection for intraocular lymphoma.

Risk Factors

  • Common risk factors in the development of intraocular lymphoma, include:[3]
  • Toxoplasma gondii 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.[2]
  • 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

Symptoms

  • Symptoms of intraocular lymphoma may include the following:[2]
  • Burning of the eye
  • Redness of the eye
  • Blurred vision
  • Photophobia or sensitivity to light
  • Eye pain
  • Floaters (which are dark spots that float in the visual field)
  • Headache
  • Severe symptoms of intraocular lymphoma, may include:[2]

Physical Examination

  • Patients with intraocular lymphoma usually appear pale or malnourished.[2]
  • 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.[2]

Imaging Findings

  • Magnetic resonance image (MRI) is the imaging modality of choice for patients with intraocular lymphoma.[2]
  • 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.[2]
  • Other medical therapies for intraocular lymphoma, may include:[2]
  • Methotrexate
  • Intravitreal rituximab
  • Localized external beam radiation therapy (EBRT)

Surgery

  • Surgery is the mainstay of therapy for intraocular lymphoma.

Prevention

  • There are no primary preventive measures available for intraocular lymphoma.[2]
  • 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.[2]

References

  1. 1.0 1.1 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. 2.00 2.01 2.02 2.03 2.04 2.05 2.06 2.07 2.08 2.09 2.10 2.11 2.12 2.13 2.14 2.15 2.16 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
  3. 3.0 3.1 3.2 3.3 3.4 3.5 3.6 3.7 Intraocular lymphoma. Wikipedia. https://en.wikipedia.org/wiki/Intraocular_lymphoma

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