Lymphoma is a cancer of the lymphatic system. There are 2 major kinds of lymphomas: non-Hodgkin lymphoma (NHL) and Hodgkin lymphoma (HL). Ocular lymphomas are almost always non-Hodgkin lymphoma (Hodgkin lymphoma very rarely affects the eye). Primary ocular lymphoma is the second most common type of primary eye cancer in adults, but it is an uncommon cancer. A non-Hodgkin lymphoma or leukemia can also spread (metastasize) to the eye (secondary intraocular lymphoma).
Ocular lymphoma can occur within the eye (intraocular) or can affect the orbit or adnexal structures, including the lacrimal gland, eyelid and conjunctiva. Intraocular lymphoma is rare. Lymphoma of the orbit is the most common orbital tumour in adults.
Most people who develop ocular lymphoma are elderly or have a weakened immune system.
The signs and symptoms of ocular lymphoma are similar to signs and symptoms of other eye cancers and include:
Although ocular lymphoma often affects both eyes, it can cause more symptoms in one eye.
Many of the same tests used to diagnose and stage any type of eye cancer are also used to diagnose and stage ocular lymphoma.
The grade of ocular lymphoma is based on how slowly or quickly the tumour is growing and how it is likely to behave.
Different types of NHL can occur in the eye and its structures. Most ocular lymphomas are B-cell lymphomas.
In 30%–35% of cases, lymphomas of the orbit and adnexal structures are associated with lymphoma elsewhere in the body (systemic lymphoma).
Ocular lymphomas do not have their own staging system. They may be staged using the same staging system for non-Hodgkin lymphoma. Pathologists are working on developing a clinical staging system for orbital and adnexal lymphomas.
Prognosis depends on the location, stage and grade of ocular lymphoma. Intraocular lymphomas are often high-grade tumours. Lymphomas of the orbit and adnexal structures are often low-grade.
The outcome is better if the lymphoma is confined to the orbit and adnexal structures. Lymphomas that spread to the central nervous system (CNS) or other parts of the body have a poorer prognosis.
Treatment of ocular lymphoma depends on the particular subtype of lymphoma and whether it is confined to the eye or is in other parts of the body. Treatment may include external beam radiation therapy, chemotherapy or a combination of both (chemoradiation).
Chemotherapy drugs may be given into a vein (intravenous) or into the eye (intraocular). If ocular lymphoma has spread to the brain or spinal cord, drugs can be given directly into the cerebrospinal fluid (intrathecal chemotherapy).
Biological therapies may also be a treatment option for ocular lymphoma, depending on the particular lymphoma subtype.
For more information on treatment options, go to non-Hodgkin lymphoma.
Follow-up after ocular lymphoma treatment varies. People with ocular lymphoma should talk to their doctor about a follow-up plan that suits their individual situation. Ocular lymphoma tends to recur, especially within the first 2 years after treatment. People with ocular lymphoma may have more frequent follow-up visits during this time.
Tests may be ordered as part of follow-up or if the doctor suspects the cancer has come back (has recurred). Follow-up tests may include:
If a recurrence is found during follow-up, the oncology team will assess the person with cancer to determine the best treatment options.
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