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Mantle cell lymphoma in the ocular adnexal region.

Abstract：

PURPOSE:To study the clinicopathologic features of mantle cell lymphoma (MCL) in the ocular adnexal region. DESIGN:Retrospective review. METHODS: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. RESULTS: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%. CONCLUSIONS: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.

journal_name

Ophthalmology

journal_title

Ophthalmology

authors

Looi A,Gascoyne RD,Chhanabhai M,Connors JM,Rootman J,White VA

doi

10.1016/j.ophtha.2004.07.025

subject

Has Abstract

pub_date

2005-01-01 00:00:00

pages

114-9

issue

eissn

0161-6420

issn

1549-4713

pii

S0161-6420(04)01268-0

journal_volume

112

pub_type

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Mantle cell lymphoma in the ocular adnexal region.