Intraocular lymphoma
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:
- Primary cerebral lymphoma
- Primary leptomeningeal lymphoma
- Primary intraocular lymphoma
- Primary spinal lymphoma
- Neurolymphomatosis
| {{{ A10 }}} | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| {{{ B10 }}} | {{{ B11 }}} | ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| {{{ C10 }}} | {{{ C11 }}} | {{{ C12 }}} | {{{ C13 }}} | {{{ C14 }}} | {{{ C15 }}} | ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| {{{ D10 }}} | {{{ D11 }}} | ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
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.[4]
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
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
- 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
- ↑ 1.0 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.
- ↑ 2.00 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 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
- ↑ 3.0 3.1 3.2 3.3 3.4 3.5 3.6 Intraocular lymphoma. Wikipedia. https://en.wikipedia.org/wiki/Intraocular_lymphoma
- ↑ 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.
- ↑ 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.