Intraocular Lymphoma

This entry looks at intraocular lymphoma, known as primary intraocular lymphoma (PIOL), one of the subtypes of lymphoma. 'Lymphoma' is an umbrella term that loosely refers to several dozen independent categorical types and subtypes of cancers of the lymphatic system.

When a primary lymphoma is found in the eye (an 'ocular lymphoma), it can be categorized as either:

-- Intraocular lymphoma, meaning it is developing within the eye

or

-- Orbital adnexal lymphoma, meaning it is developing in the areas around the eye

Intraocular lymphoma can be of B-cell or T-cell origin. It can also be a secondary site, and therefore be considered a subtype of primary central nervous system lymphoma (PCNSL), and this is in fact the biggest concern with intraocular lymphoma—that it is found to have spread from the central nervous system (CNS). Prognosis in these cases is very poor. Typically, if a diagnosis of intraocular lymphoma is made, then it is followed by a complete neurological work-up to see if there is any CNS involvement or not.

Symptoms of Intraocular Lymphoma

Symptoms of PIOL include:

-- Decreased vision
-- Vitreous floaters (you know, those little fibers and shapes we kind of see in the corners of our eyes but can never actually look at. They're common in us all, but if there is a big increase, then it's a concern. Vitreous is a substance that makes up most of the eye; as it shrinks, floaters form).
-- Uveitis (swelling of the middle layer of the eye, the uvea)

Treatment for Intraocular Lymphoma

If the disease is localized—meaning it has not spread to distant sites in the body, then chemotherapy loses its appeal as an effective treatment because chemotherapy drugs go everywhere in the body, and if the disease is only in the eye, the odds of getting good distribution of the chemo in that area are not good. Rather, the preferred treatment is known as external beam radiation, and the effective doses are considered to be on the low end, which is good.

Sometimes chemotherapy is used after radiation, as a means of 'mopping up' any remaining cancer cells missed by the radiation.

The 5 year overall survival rate for intraocular lymphoma is said to be around 60%.

Sources

MedScape, Ocular lymphoma

Karma A et al. Primary intraocular lymphoma: improving the diagnostic procedure. Opthamology 2007 Jul;114(7):1372-7.

Eye Cancer Network, Intraocular lymphoma

National Eye Institute, Facts about floaters