Represents a small number of NHL’s, about 6% of all extranodal NHL’s 7% of nongastric marginal zone lymphomas.
Make up 26% of ocular adnexal tumors.
Diagnosis based on histological examination combined with immunohistochemical analysis and molecular analysis.
Biopsy depends on site of lesion and may involve the conjunctiva, eyelid, orbit or lacrimal gland.
Majority of lesions, 95-100% of cases are B cell lymphomas and 80% are low grade.
Can be the initial presentation of lymphoma or may represent a disseminated NHL.
May present with proptosis, diplopia, ptosis and conjunctival changes.
Lacrimal gland constitutes approximately 20% ocular lymphomas.
These lesions may spread locally or disseminate.
High incidence of marginal zone B cell lymphoma or mucosa associated lymphoid tissue lymphoma (MALT) reported in approximately 50% of cases.
Extranodal marginal zone lymphomas of mucosal associated lymphoid tissue account for 35-90% of lesions and follicular and diffuse large cell lymphomas each account for approximately 10%.