Table of ContentsView AllTable of ContentsTypesSymptomsCausesDiagnosisTreatment

Table of ContentsView All

View All

Table of Contents

Types

Symptoms

Causes

Diagnosis

Treatment

Eye lymphoma, also called ocular lymphoma, is a type of eye cancer. It is the most common type of malignant eye tumor. The condition may cause eye redness or decreased vision, and it can advance to result in eye damage and blindness. While anyone can develop lymphoma of the eye, having an immune deficiency is a risk factor.

Definitive diagnosis relies on a biopsy of the eye, which is a delicate procedure. Treatment to prevent progression includes surgery,chemotherapy, and radiation therapy.

Types of Ocular Lymphoma

PIOL/Vitreoretinal Lymphoma

This ocular lymphoma affects theretina(the area in the back of the eye that senses light and converts it to signals for the brain to interpret), the vitreous of the eye (a jellylike substance that fills most of the eyeball), or theoptic nerve(the nerve that detects visual input).

PIOL is considered a central nervous system lymphoma because it arises from structures in the eye that are part of the nervous system.

This tumor is usually aggressive and often spreads to the brain.

Uveal lymphoma

This tumor involves the uvea, which is the part of the eye that’s directly beneath thesclera(the white of the eye). Uveal lymphoma can affect the choroid (blood vessels of the eye), theiris(the colored part around thepupil), or the ciliary body (muscles and other structures around the iris).

This type of lymphoma is often designated aslow gradebecause it doesn’t have a tendency to be very aggressive.

Ocular Adnexal Lymphoma

This type of lymphoma begins in structures that are near the eye but are outside the eye itself. Ocular adnexal lymphoma involves the orbit (eye socket), theconjunctiva(lining of the eye), or the lacrimal gland (a structure that makes tear ducts), or theeyelid.

There areother types of eye cancerthat aren’t lymphomas, such asretinoblastomaand eye melanoma. Non-lymphoma eye cancers have different symptoms, causes, prognosis, and treatments than ocular lymphoma.

The Eye Lens' Function and Structure

Ocular Lymphoma Symptoms

The early symptoms of ocular lymphoma are fairly non-specific and can be the same as signs of eye infections, degenerative eye diseases, or inflammation of the eye. The most common early symptoms involve subtle vision changes and mild to moderate eye discomfort.

Eye lymphomas can start off in one eye, but they generally eventually affect both eyes.

Symptoms can include:

You might experience the same effects in both eyes, but they can be more noticeable in one eye than the other. It’s also possible for each eye to have different effects of the disease.

Advanced Tumors

Ocular lymphomas can enlarge, causing more noticeable effects and complications. Large tumors may cause symptoms due to pressure on the eyeball.

An advanced eye tumor may:

Lymphoma, including lymphoma of the eye, occurs when genetic changes inDNAalter the behavior of immune cells.These mutations cause the cells to become dysfunctional in terms of their role in immunity. The alterations also cause the cells to multiply and spread more than usual.

Generally, eye lymphomas form a tumor mass in or around the eye.

Cancer Cells vs. Normal Cells: How Are They Different?

Risk Factors

Eye lymphomas can develop without any risk factors, but immune diseases, immune deficiency, or a history of chemotherapy or radiation can increase the chances of developing ocular lymphoma.

These risk factors can lead to the aforementioned mutations. If a cell that’s had a mutation survives, it can lead to cancer.

A tumor in the eye can be diagnosed based on anon-invasive eye examination, imaging studies, and a biopsy of the tumor in the eye. Your medical assessment would also include an evaluation of whether the lymphoma is present elsewhere in your body.

Classification of your tumor involves a determination of the tumor type, grade, and stage.

Lymphoma can be eitherHodgkin’sornon-Hodgkin’stype. Most ocular lymphomas are classified as non-Hodgkin’s tumors. This differentiation is based on certain characteristics that are seen on a biopsy evaluation. Generally, non-Hodgkin’s tumors are more aggressive than Hodgkin’s tumors.

Biopsy

A biopsy for the diagnosis of an eye tumor is a surgical procedure in which a sample of tissue is taken from the eye. The surgery requires minimal excision of tissue to avoid harming the eye or causing any impairment of vision.

Generally, your sample will be sent to a laboratory right away, but full results might not be available for several days.

Your healthcare provider will give you instructions regarding eye care after your biopsy.

Ocular lymphomas don’t improve on their own. Because of the nonspecific nature of primary intraocular lymphoma presentation, the condition is a diagnostic challenge. The prognosis for this condition remains poor with a five-year mortality of less than 25%.

Chemotherapy is considered systemic therapy, and it treats the primary tumor, as well as metastatic lesions. If there is central nervous system involvement, systemic treatment is recommended.

External beam radiation therapy (EBRT) is considered a local treatment that can be directed to target the tumor in the eye specifically, or may also be used for treating brain metastasis to make the tumor shrink.

Each tumor is treated based on its classification:

Side effects of treatment with external beam radiation can include:

Managing the Effects of Radiation Therapy

Many of these side effects can cause a spectrum anywhere from discomfort to total loss of vision. However, the side effects of ocular lymphoma treatment are not as harmful as untreated ocular lymphoma.

A Word From Verywell

Eye lymphomas are very rare. These tumors can cause many of the same symptoms as more common eye diseases. This is why it is important to have regularly scheduled eye examinations and to see your healthcare provider if you develop any eye symptoms or problems related to your vision. Early diagnosis increases the chances of a good outcome.

9 SourcesVerywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.American Academy of Ophthalmology.What is eye cancer?Farrall AL, Smith JR.Eye involvement in primary central nervous system lymphoma.Surv Ophthalmol. 2020;65(5):548-561. doi:10.1016/j.survophthal.2020.02.001Aronow ME, Portell CA, Sweetenham JW, Singh AD.Uveal lymphoma: clinical features, diagnostic studies, treatment selection, and outcomes.Ophthalmology.2014;121(1):334-341. doi:10.1016/j.ophtha.2013.09.004American Academy of Ophthalmology.Demystifying ocular lymphoma.White VA.Understanding and classification of ocular lymphomas.Ocul Oncol Pathol. 2019;5(6):379-386. doi:10.1159/000499845American Cancer Society.What is eye cancer?American Academy of Ophthalmology.What is ocular lymphoma?Tang LJ, Gu CL, Zhang P.Intraocular lymphoma.Int J Ophthalmol. 2017;10(8):1301-1307. Published 2017 Aug 18. doi:10.18240/ijo.2017.08.19Tanenbaum RE, Galor A, Dubovy SR, Karp CL.Classification, diagnosis, and management of conjunctival lymphoma.Eye Vis (Lond). 2019;6:22. doi:10.1186/s40662-019-0146-1

9 Sources

Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.American Academy of Ophthalmology.What is eye cancer?Farrall AL, Smith JR.Eye involvement in primary central nervous system lymphoma.Surv Ophthalmol. 2020;65(5):548-561. doi:10.1016/j.survophthal.2020.02.001Aronow ME, Portell CA, Sweetenham JW, Singh AD.Uveal lymphoma: clinical features, diagnostic studies, treatment selection, and outcomes.Ophthalmology.2014;121(1):334-341. doi:10.1016/j.ophtha.2013.09.004American Academy of Ophthalmology.Demystifying ocular lymphoma.White VA.Understanding and classification of ocular lymphomas.Ocul Oncol Pathol. 2019;5(6):379-386. doi:10.1159/000499845American Cancer Society.What is eye cancer?American Academy of Ophthalmology.What is ocular lymphoma?Tang LJ, Gu CL, Zhang P.Intraocular lymphoma.Int J Ophthalmol. 2017;10(8):1301-1307. Published 2017 Aug 18. doi:10.18240/ijo.2017.08.19Tanenbaum RE, Galor A, Dubovy SR, Karp CL.Classification, diagnosis, and management of conjunctival lymphoma.Eye Vis (Lond). 2019;6:22. doi:10.1186/s40662-019-0146-1

Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.

American Academy of Ophthalmology.What is eye cancer?Farrall AL, Smith JR.Eye involvement in primary central nervous system lymphoma.Surv Ophthalmol. 2020;65(5):548-561. doi:10.1016/j.survophthal.2020.02.001Aronow ME, Portell CA, Sweetenham JW, Singh AD.Uveal lymphoma: clinical features, diagnostic studies, treatment selection, and outcomes.Ophthalmology.2014;121(1):334-341. doi:10.1016/j.ophtha.2013.09.004American Academy of Ophthalmology.Demystifying ocular lymphoma.White VA.Understanding and classification of ocular lymphomas.Ocul Oncol Pathol. 2019;5(6):379-386. doi:10.1159/000499845American Cancer Society.What is eye cancer?American Academy of Ophthalmology.What is ocular lymphoma?Tang LJ, Gu CL, Zhang P.Intraocular lymphoma.Int J Ophthalmol. 2017;10(8):1301-1307. Published 2017 Aug 18. doi:10.18240/ijo.2017.08.19Tanenbaum RE, Galor A, Dubovy SR, Karp CL.Classification, diagnosis, and management of conjunctival lymphoma.Eye Vis (Lond). 2019;6:22. doi:10.1186/s40662-019-0146-1

American Academy of Ophthalmology.What is eye cancer?

Farrall AL, Smith JR.Eye involvement in primary central nervous system lymphoma.Surv Ophthalmol. 2020;65(5):548-561. doi:10.1016/j.survophthal.2020.02.001

Aronow ME, Portell CA, Sweetenham JW, Singh AD.Uveal lymphoma: clinical features, diagnostic studies, treatment selection, and outcomes.Ophthalmology.2014;121(1):334-341. doi:10.1016/j.ophtha.2013.09.004

American Academy of Ophthalmology.Demystifying ocular lymphoma.

White VA.Understanding and classification of ocular lymphomas.Ocul Oncol Pathol. 2019;5(6):379-386. doi:10.1159/000499845

American Cancer Society.What is eye cancer?

American Academy of Ophthalmology.What is ocular lymphoma?

Tang LJ, Gu CL, Zhang P.Intraocular lymphoma.Int J Ophthalmol. 2017;10(8):1301-1307. Published 2017 Aug 18. doi:10.18240/ijo.2017.08.19

Tanenbaum RE, Galor A, Dubovy SR, Karp CL.Classification, diagnosis, and management of conjunctival lymphoma.Eye Vis (Lond). 2019;6:22. doi:10.1186/s40662-019-0146-1

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