Orbital Lymphomas

Want to submit your case? Click HERE

Orbital lymphoma is a rare tumour; it represents the second type of primary adult eye tumours, while it is associated with systemic lymphoma in 30%-35% of cases. Orbital tumours are lesions that develop in the tissues surrounding the eye globe and are divided into:

• Primary, that is, they originate in the orbit
• Secondary, that is, they originate in adjacent regions such as the eyeball, the paranasal sinuses, nasopharynx, lacrimal glands, etc.
• Metastatic, that is, they originate in areas distant from the orbit such as the breast, lung, colon, kidney, etc.

Lymphoma is the most frequently occurring malignant tumour of the orbit in adults.There are two main types of lymphoma:

• Hodgkin lymphoma (HL)
• Non-Hodgkin lymphoma (NHL)

Orbital lymphoma is usually of the non-Hodgkin type, as ocular involvement in Hodgkin lymphoma is very rare. Non-Hodgkin orbital lymphomas of B-cells lineage are more frequent. They can be classified among the MALT (mucosa-associated lymphoid tissue) lymphomas, and are characterised by their low aggressiveness, slow growth and high recurrent rates.

Causes of orbital lymphomas

Women in the 50- to 70-year age group are most often affected by orbital lymphomas.This type of tumour commonly affects the most debilitated individuals and/or those with compromised immune systems, such as:

• The elderly
• Individuals with diseases that weaken the immune system, such as AIDS
• Individuals taking anti-rejection drugs following a transplant

Although at present there are no certain causes of orbital lymphomas besides the risk factors previously mentioned, according to recent studies, it seems that some infectious agents may play a key role in the pathogenesis of eye lymphomas, including those of the orbit. In fact, it seems that repeated and persistent infection with Chlamydia Psittaci is associated to the development of these tumours. C. Psittaci is the bacterial agent that causes Psittacosis, a human lung infection caused by exposure to infected birds.

Symptoms of orbital lymphomas

The main symptoms of orbital lymphoma are related to vision, although they may be common to other much less serious eye conditions.
Some of main symptoms are:

• Blurred vision
• Decreased vision
• Diplopia
• Eye redness
• Photophobia
• Ocular motility disturbances
• Bulging of eyeball

Diagnosis of orbital lymphomas

For a correct diagnosis of ocular tumours, a visit to the ophthalmologist is priority. After an accurate medical history of the patient, the ophthalmologist carries out a specialised examination. After analysing the eye in full, both inside and outside, and checking vision, eye movements and blood vessels, the ophthalmologist makes the diagnosis or prescribes further examinations.

Once the orbital lymphoma is diagnosed, further tests aimed at verifying whether the disease is also present in other anatomical regions are required.

Treatment of orbital lymphomas

Treating orbital lymphomas involves a multidisciplinary approach, but the choice of therapy may vary based on the type of lymphoma, its location (that is, if it affects only the orbit or other parts of the body as well) and the patient's general medical condition.

Treatment can involve chemotherapy, radiotherapy or a combination of both. If the lymphoma has spread to the brain or spinal cord, chemotherapy medication can be administered directly into the cerebrospinal fluid.