Mantle cell lymphoma in the ocular adnexal region

To study the clinicopathologic features of mantle cell lymphoma (MCL) in the ocular adnexal region.

Retrospective review.

The slides of 23 suspect patients were reevaluated with a panel of monoclonal antibodies, including anti-CD20, cyclin-D1, CD5, CD3, and p53 immunostains. Patients confirmed to have MCL were examined retrospectively on the basis of chart review.

Ten patients with periocular MCL were included in the study on the basis of characteristic histopathologic features and coexpression of nuclear cyclin-D1. This included 1 female and 9 male patients, with an age range of 32 to 84 years (median, 73.5 years). Median follow-up was 20 months (range, 5-172 months). Six of the 10 patients died, all of lymphoma. The orbit (90%) was most commonly involved followed by the lacrimal gland (50%) and lid (50%), with 90% of cases having lymphoma present at 2 or more periocular sites. Most had a primary periocular presentation (80%) that was associated with stage III/IV disease (80%), including atypical cells in the peripheral blood smear (60%) and bone marrow involvement (70%) at presentation. Three cases were CD5-negative, and 2 other cases showed composite histologic findings (MCL and follicular lymphoma and MCL and a plasma cell neoplasm). Fluorescent in situ hybridization performed in these 2 cases demonstrated t(11;14) in the MCL component. Actuarial survivals were median progression-free (PFS) survival, 12 months; median overall survival (OS), 57 months; 5-year PFS, 0; 5-year OS, 39%.

Mantle cell lymphoma presenting in the ocular adnexal region has a male predominance and tends to affect an elderly age group, as is typical of MCL involving nodal sites. A higher frequency of these tumors fail to co-express CD5, and composite lymphomas were observed in 20% of patients. Mantle cell lymphoma presenting in the ocular adnexal region is associated with advanced-stage disease and short PFS but an OS similar to MCL at other sites.