Rare entity comprising a small proportion of orbital neoplasms and non-Hodgkin’s lymphomas.
Incidence highest among Asians/Pacific Islanders, and lower in whites and lower yet in blacks.
Equal among the genders.
Annual increases in incidence of approximately 6%.
MALTomas the most common subtype of ocular adnexal lymphoma.
Primary management consists of radiation, with the addition of chemotherapy depending upon stage.
Median age about 57 years (Russell W et al).
Approximately 80% of patients for stage IAE.
Less than 20% are stage IVAE.
Greater than 43% have conjunctiva involvement, 25% of cases involve orbital structures, greater than 18% involve the lacrimal duct or sac, , and 12 1/2% involve more than one orbital subsite (Russell W et al).
Radiation alone leads to 85% complete remission and 75% local control.