Intraocular lymphoma
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]
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]
- 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
- Ocular metastasis (most common)
- Choroidal hemangioma
- Vitrous lymphoma
- Retrolental fibroplasia
Epidemiology and Demographics
Prevalence
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
- 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
- Burning of the eye
- Redness of the eye
- Visual field defect
- Blurred vision
- Photophobia or sensitivity to light
- Eye pain
- Floaters
- Headache
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
- ↑ 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.
- ↑ 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.
- ↑ 3.0 3.1 3.2 3.3 3.4 3.5 Intraocular lymphoma. Wikipedia. https://en.wikipedia.org/wiki/Intraocular_lymphoma
- ↑ 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
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ Chan CC (2003). "Molecular pathology of primary intraocular lymphoma". Trans Am Ophthalmol Soc. 101: 275–92. PMC 1358994. PMID 14971583.
- ↑ 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.