cyclin-d1 and Eyelid-Neoplasms

Topics: Antigens, CD20; Antirheumatic Agents; CD5 Antigens; Cyclin D1; Eyelid Neoplasms; Eyelids; Herpesvirus 4, Human; Humans; Lymphoma, Large B-Cell, Diffuse; Male; Methotrexate; Middle Aged; Neoplasm Regression, Spontaneous

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.

Topics: Adult; Aged; Aged, 80 and over; Antigens, CD; Antineoplastic Combined Chemotherapy Protocols; Biomarkers, Tumor; Combined Modality Therapy; Cyclin D1; Eye Neoplasms; Eyelid Neoplasms; Female; Follow-Up Studies; Humans; Immunohistochemistry; Lacrimal Apparatus Diseases; Lymphoma, Mantle-Cell; Male; Middle Aged; Orbital Neoplasms; Retrospective Studies; Survival Rate; Tumor Suppressor Protein p53

The organization of immunoglobulin heavy chain (IgH), light chain (kappa and lambda) and T cell receptor (TCR) beta chain gene loci in 10 patients with ocular adnexal pseudolymphoma was investigated. Eight of them showed IgH gene rearrangement in at least one of the 3 restriction enzymes-digested DNAs extracted from ocular adnexal neoplasms. In contrast, none of them exhibited clonal TCR beta chain gene rearrangement. The configuration of bcl-1, bcl-2 and c-myc oncogenes was also studied by Southern blot technique. Two patients had a rearranged joining region, IgH-containing fragment that comigrated with the rearranged bcl-1 fragment. C-myc gene rearrangement was found in only one patient who also had bcl-1 gene rearrangement. In ocular adnexal pseudolymphoma, none demonstrated bcl-2 gene rearrangement; however, in bone marrow cells, one patient with systemic lymphadenopathy exhibited both IgH and bcl-2 gene rearrangements. Three genotypic subsets of these ocular adnexal pseudolymphoma can be identified by the configuration of IgH gene and related oncogenes: with germline configuration of IgH gene and bcl-1, bcl-2 and c-myc oncogenes; with rearrangement of IgH gene but germline configuration of these oncogenes; and with recombination between rearranged IgH and bcl-1 genes. These results suggest in ocular adnexal pseudolymphoma a spectrum of clonal change evolving from polyclonal to monoclonal B-population, and further to monoclonal B-population with rearranged bcl-1, c-myc and/or bcl-2 oncogenes.

Topics: Adult; Aged; Aged, 80 and over; Cyclin D1; DNA, Neoplasm; Eyelid Neoplasms; Gene Rearrangement; Genes, Immunoglobulin; Genes, myc; Humans; Lymphoma; Male; Middle Aged; Orbital Neoplasms; Proto-Oncogene Proteins; Proto-Oncogene Proteins c-bcl-2; Receptors, Antigen, T-Cell; Receptors, Antigen, T-Cell, alpha-beta