Vitreoretinal Lymphoma (Intraocular Lymphoma): Signs, Diagnosis and Treatment

Vitreoretinal lymphoma is a rare cancer of lymphocytes — a type of white blood cell — that grows in the retina (the light-sensing layer at the back of the eye) and in the vitreous, the clear gel that fills the eye. It is also called primary intraocular lymphoma or ocular lymphoma. Its first signs, such as floaters and misty vision, closely resemble inflammation inside the eye (uveitis), so many people are treated for uveitis for months before the real cause is found.

This page explains how the diagnosis is confirmed and how eye treatment is combined with brain and body care, with short notes on lymphoma around the eye and on leukemia. It is part of our eye cancer guide.

What is vitreoretinal lymphoma?

Most cases are primary vitreoretinal lymphoma, a form of primary central nervous system lymphoma that first appears in the eye. The retina develops as an outgrowth of the brain, and the two are closely linked: in published series, 35–90% of patients develop lymphoma in the brain or spinal fluid at some point. Almost all cases are diffuse large B-cell lymphoma, an aggressive type. Other forms of eye lymphoma behave differently:

Who gets it?

Vitreoretinal lymphoma accounts for less than 1% of all tumors inside the eye. It is usually diagnosed in middle or later adult life, slightly more often in women, and in 80–90% of patients both eyes are eventually affected. It is not contagious and not inherited.

Why is it called a masquerade syndrome?

A masquerade syndrome is a disease — often a cancer — that imitates ordinary inflammation. Typical symptoms of vitreoretinal lymphoma are:

• floaters — spots or cobwebs drifting across your vision;
• blurred or hazy vision, often in both eyes;
• often little pain or redness, unlike many types of uveitis.

The specialist sees cells floating in the vitreous and sometimes yellowish patches beneath the retina. Steroid drops, tablets or injections may seem to help for a while, but the haze returns.

How is vitreoretinal lymphoma diagnosed?

Scans such as optical coherence tomography (OCT) can show deposits in or under the retina, but proof requires testing fluid from inside the eye:

1. Examination and imaging of both eyes, with a review of earlier treatment — especially the response to steroids.
2. Vitreous biopsy (diagnostic vitrectomy) — a short operation to take a sample of the vitreous gel, planned so that several tests can be run on a small volume.
3. Laboratory analysis of the sample (see the table below).
4. Staging — brain MRI with contrast and a lumbar puncture to examine the cerebrospinal fluid, organized with the hemato-oncology team, who may add other tests.
5. A joint treatment plan agreed by the eye specialist and the lymphoma team.

Because steroids can make lymphoma cells harder to find, your specialist may advise on steroid timing before the biopsy — never stop a prescribed medicine on your own. See also how eye tumors are diagnosed.

How is intraocular lymphoma treated?

Treatment aims to clear the lymphoma from the eye, protect sight and control — or watch for — disease in the brain and body. The ocular oncologist plans the eye treatment; hemato-oncology specialists plan the rest.

The optimized methotrexate schedule described in 2024 uses weekly injections for 4 weeks, then injections every 2 weeks for 2 months and monthly for 6–12 months. Its main side effect, irritation of the corneal surface (keratopathy), occurred in about 10% of eyes, compared with 20–30% on older schedules. Whether lymphoma confined to the eyes also needs systemic treatment is still debated and is decided with the lymphoma team.

What is the outlook?

The outlook depends mainly on whether the brain becomes involved. Eye treatment is usually effective, but lymphoma can return in either eye or appear in the brain later, so follow-up with eye examinations and periodic brain MRI is lifelong. Survival in primary central nervous system lymphoma has improved from about 1 year historically to more than 3 years with modern chemotherapy, and depends on the molecular subtype: about 70% of patients with the "germinal center" type were disease-free at 5 years, against about 30% with the "activated B-cell" type. Your hemato-oncologist can explain what these group figures mean for you; see also is eye cancer curable?

Lymphoma around the eye: the "salmon patch"

Lymphoma can also grow in the conjunctiva (the membrane over the white of the eye), the orbit, the eyelid or the tear gland and tear sac — together called ocular adnexal lymphoma. Most are slow-growing marginal zone (MALT) lymphomas. On the conjunctiva, lymphoma typically forms a smooth, salmon-pink patch that usually does not hurt, often in the fold between eyelid and eyeball. A biopsy is essential, because harmless reactive lymphoid hyperplasia can look identical; part of the tissue is sent fresh for flow cytometry.

In a Wills Eye series, lymphoma elsewhere in the body was found in 17% of patients with a conjunctival tumor in one eye and 47% when both eyes were affected, so staging with the hematology team follows. Localized disease is usually treated with low-dose radiotherapy, increasingly with very low doses of 4 Gy; rituximab, doxycycline for selected cases and chemo-immunotherapy for widespread disease are alternatives. Five-year lymphoma-specific survival for the marginal zone type was about 97%. See conjunctival tumors and orbital tumors.

Can leukemia affect the eye?

Leukemia, a cancer of the blood-forming cells, can occasionally infiltrate eye tissues, including the iris. Anterior segment OCT can document such infiltration in detail, a topic on which Prof. Türkoğlu has published. If you are being treated for leukemia and notice a change in the appearance of an eye, pain or blurred vision, tell your hematology team and arrange an eye examination.

Diagnosis and treatment planning in Antalya

This disease sits between eye care and cancer care. Prof. Türkoğlu evaluates the eye findings, reviews the course of the "uveitis" and earlier scans, plans the diagnostic steps including the biopsy, and follows the eye's response to treatment. Brain imaging, spinal fluid tests and systemic treatment are organized with hemato-oncology specialists.

From abroad, you can start with a remote second opinion by sending OCT scans, retinal photographs, a list of steroid treatments with dates, laboratory reports and brain MRI reports. Because injections continue for months, part of the plan may be shared with doctors near your home, decided case by case. See also international patients and our page for referring doctors.