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:
- double, blurred or decreased vision
- seeing floaters
- redness or swelling of the eye
- sensitivity to light
- painless bulging of the eye out of the orbit (called proptosis) – occurs with orbital tumours
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.
- medical history and physical examination
- Physical exams include a complete eye examination and neurologicalneurologicalHaving to do with nerves or the nervous system. examination.
- ophthalmic ultrasound
- magnetic resonance imaging (MRI) of the eye and orbit
- MRI is also used to look for lymphoma in the brain or meningesmeningesThe membranes that cover and protect the brain and spinal cord..
- computed tomography (CT) scan of the eye and orbit
- CT scan is also used to check for lymphoma in other parts of the body.
- vitreous biopsy (vitrectomy)
- This test is used to diagnose intraocular lymphoma.
- lumbar puncture (LP)
- Lumbar puncture is used to find out if ocular lymphoma has spread to the cerebrospinal fluid (CSF) or brain.
- bone marrow aspiration and biopsy
- This test may be done to stage lymphoma that has spread beyond the eye into the bone marrow.
- positron emission tomography (PET) scan
- A PET scan may be done to identify areas in the body where ocular lymphoma has spread.
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.
- Extranodal marginal zone of mucosa-associated lymphoid tissue (MALT lymphoma) accounts for most orbital and adnexal lymphomas. The other types of lymphoma that can occur are follicular lymphoma, mantle cell lymphoma and diffuse large B-cell lymphoma. These types occur less often than MALT lymphomas.
- Intraocular lymphoma often occurs with another type of lymphoma called primary central nervous system (CNS) lymphoma. Intraocular lymphoma also tends to spread to the brain, spinal cord or meninges.
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:
- CT scan
- lumbar puncture
If a recurrence is found during follow-up, the oncology team will assess the person with cancer to determine the best treatment options.