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

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Intraocular lymphoma is a rare subtype of primary central nervous system lymphoma. Intraocular lymphoma may affect the eye secondarily from 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 are 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

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

## Classification

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

* Retinal
* Retinal
* Vitreoretinal
* Vitreal
* Uveal
* Iridal
* Ciliary
* Choroidal

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| Retinal|

| Uveal|

Retinal|

| Vitreoretinal|

| Vitreal|

| Iridal|

| Ciliary|

| Choroidal|

| Primary|

| Secondary

## Pathophysiology

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* 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]

* C-MYC gene
* CCND1 gene

* 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

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* There are no established causes of intraocular lymphoma.

## Differentiating Intraocular Lymphoma from Other Diseases

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

* Ocular metastasis (most common)
* Pigmented nevi
* Congenital hypertrophy of the retinal pigment epithelium
* Optic disc melanocytoma
* Hypertrophy of the retinal pigment epithelium
* Hyperplasia of retinal pigment epithelium
* Choroidal hemangioma
* Choroidal metastasis
* Choroidal osteoma
* Peripheral exudative hemorrhagic chorioretinopathy
* Choroidal hemorrhage
* Hemorrhagic detachment of retina and retinal pigment epithelium
* Posterior nodular scleritis
* Intraocular leiomyoma
* Adenoma of retinal pigment epithelium
* Retinoblastoma
* Uveal metastases
* Choroidal detachment
* Choroidal cyst
* Uveal neurofibroma
* Uveal schwannoma
* Choroidal hemangioma
* Vitrous lymphoma
* Retrolental fibroplasia

## Epidemiology and Demographics

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### Prevalence

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

### Age

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

### Gender

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

### Race

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* There is no racial predilection for intraocular lymphoma.

## Risk Factors

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* Common risk factors in the development of intraocular lymphoma, include:[7][8]

* Toxoplasma gondii infection
* EBV infection
* HHV8 infection

## Natural History, Complications and Prognosis

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* 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

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### History and Symptoms

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

* Burning of the eye
* Redness of the eye
* Visual field defect
* Blurred vision
* Photophobia or sensitivity to light
* Eye pain
* Floaters
* Headache

### Physical Examination

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* 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

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

### Imaging Findings

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* 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

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### Medical Therapy

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* 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

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* Surgery is the mainstay of therapy for intraocular lymphoma.

### Primary Prevention

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* 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

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## References

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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.