Conjunctival Lymphoma
All about conjunctival lymphoma
Conjunctival lymphoma is a malignant tumor derived from a monoclonal proliferation of immune system cells called lymphocytes. These are usually B lymphocytes that transform into lymphomatous cells. It is always a non-Hodgkin lymphoma and belongs to the family of ocular adnexal lymphomas (which can affect tissues around the eye such as the conjunctiva or the orbit, or certain intraocular tissues like the uvea). There are several subtypes of conjunctival lymphoma, with the majority being low-grade, less aggressive lymphomas (about ¾), and the rest being high-grade, more aggressive lymphomas.
Although precise statistics are not available in France, the incidence of conjunctival lymphoma is low.
Risk factors for developing conjunctival lymphoma depend on the lymphoma subtype. In general, it is a disease of adults over 50 years old.
Conjunctival involvement of ocular adnexal lymphomas usually presents as a salmon-pink conjunctival mass, which may be limited or more diffuse, unilateral or bilateral depending on the lymphoma subtype. The speed and progression of these lesions, whether slow or rapid, generally indicate the aggressiveness of the lymphomatous disease.
The proposed treatment depends on several parameters:
- whether the disease is limited to the eye or disseminated
- the patient’s age and life expectancy
- the patient’s comorbidities
- prognostic factors specific to each lymphoma subtype
In general, low-grade lymphomas are associated with a favorable prognosis, although local recurrences are frequent, whereas high-grade lymphomas are associated with a less favorable prognosis. Treatments are decided by hematology-oncology specialists and may include radiotherapy and/or immunochemotherapy.
Ophthalmologic follow-up will be determined by the referring ocular oncologist but should be long-term because local recurrences are possible, even after 5 years from initial treatment.
Systemic follow-up includes periodic imaging and laboratory tests, as recommended by the hematology-oncology specialist.