Eye Lymphoma

What is primary intraocular lymphoma?

Lymphoma is a cancer that originates in white blood cells called lymphatic cells. When lymphoma develops in the eye, it is called primary intraocular lymphoma (PIOL), and it is almost always a type of lymphoma called non-Hodgkin’s B cell lymphoma. PIOL can develop in the retina, the light sensitive tissue that lines the back of the eye; in the vitreous, the jelly-like fluid inside the eye; or in the optic nerve at the back of the eye. About 80 percent of people who have PIOL develop it in both eyes and it may also be found in the brain.

Most people who develop PIOL are elderly or have immune system diseases such as rheumatoid arthritis, AIDS (acquired immunodeficiency syndrome), or are taking medications to prevent their bodies from rejecting a transplanted organ.


What are the symptoms of primary intraocular lymphoma?

Intraocular lymphomas have a range of symptoms, making diagnosis sometimes challenging. The most common symptoms for PIOL include:

  • blurry vision
  • decrease or loss of vision
  • floaters (small dots or lines in the field of vision)
  • redness or swelling in the eye
  • increased sensitivity to light
  • eye pain (rarely)

While lymphoma usually affects both eyes, symptoms may be more obvious in one eye than the other.


How is primary intraocular lymphoma diagnosed?

Ophthalmologists diagnose intraocular lymphoma by assessing patients’ symptoms, checking vision and eye movement, examining the inside of the eye using an ophthalmoscope—an instrument with a light and a small magnifying lens, and by removing a sample of cells from the vitreous (a procedure called vitrectomy biopsy) for examination by cytopathologist.

The ophthalmologist will work with other specialists to obtain images of the brain and central nervous system to determine if the lymphoma has spread beyond the eye. They will likely also remove fluid from the spine to determine if the lymphoma has spread to the spinal fluid.


How is primary intraocular lymphoma treated?

Treatment for PIOL usually includes chemotherapy (often the drugs methotrexate and/or rituximab), which may be delivered through a vein (systemically), or directly into the eye (intravitreally) or fluid in the spine (intrathecally). The last two modes of delivering treatment enable ophthalmologists to focus the chemotherapy directly in the affected area and reduce potential side effects. 

Radiation therapy uses beams of very high energy to shrink tumors and kill cancer cells. Radiation may be targeted only at the affected eye or at both eyes, and at the brain and spinal cord. This can help prevent cancer from spreading there or help destroy hidden cancer cells.