Ocular Adnexal Extranodal Marginal Zone Research Articles

Efficacy of chemotherapy as a first-line treatment in ocular adnexal extranodal marginal zone B-cell lymphoma

BackgroundRadiotherapy is commonly used as a first-line treatment for localized ocular adnexal extranodal marginal zone B-cell lymphoma (EMZBL), despite its ophthalmologic complications. This study was undertaken to analyze the efficacy of first-line chemotherapy in treating EMZBL. Chemotherapy was followed by radiotherapy only in recurrent cases. Patients and methodsTwenty-one patients with histologically confirmed EMZBL were treated with combination of cyclophosphamide, vincristine, and prednisolone (CVP). Radiotherapy was given to CVP failure cases. ResultsCVP alone resulted in overall response rate of 100% [complete remission (CR), 76.2%]. After a median follow-up of 58 months, 14 (66.7%) of 21 cases were disease free with CVP alone, while seven cases showed disease progression, including two extra-orbital and five local failures. Radiotherapy was delivered to five local failure cases, who subsequently achieved CR with late ophthalmologic complications. There were tolerable adverse events associated with CVP. ConclusionsFront-line CVP, in conjunction with radiotherapy in recurrent cases, is effective and well tolerated in patients with localized ocular adnexal EMZBL.

Absence of Chlamydial Infection in Japanese Patients with Ocular Adnexal Lymphoma of Mucosa-Associated Lymphoid Tissue

Ocular adnexal extranodal marginal zone B-cell lymphomas of mucosa-associated lymphoid tissue (ocular adnexal MALT lymphoma) are predominately low-grade, small B-cell types and may be caused by several putative inflammatory agents. Twenty-three ocular adnexal MALT lymphoma cases, the monoclonality of which was confirmed by examination of immunoglobulin heavy chain gene rearrangement and/or cell surface antigens, were analyzed for evidence of several causative factors. A serologic evaluation of our series of patients showed no evidence of infection by Epstein-Barr virus, hepatitis C virus, or Chlamydophila psittaci. Two patients tested positive for serum antibodies for autoimmunity, and another 2 patients tested positive for antibodies against Chlamydia trachomatis. Polymerase chain reaction analysis did not reveal the presence of the chlamydial 16S ribosomal RNA (rRNA) gene or the 16S-23S spacer rRNA gene. These results indicate that the inflammatory agents in our series of ocular adnexal MALT lymphomas are still unknown and that some types of chlamydial infections are not associated with orbital adnexal MALT lymphoma in southern regions of Japan.

Fluorescence in situhybridization (FISH) analysis of primary ocular adnexal MALT lymphoma

BackgroundIt remains unknown whether primary ocular adnexal extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) is a homogeneous entity, as there are few reports of the results of cytogenetic or molecular analyses of these tumors.MethodsWe performed interphase fluorescence in situ hybridization (FISH) analysis to detect translocations and aneuploidy in 34 cases of primary ocular adnexal MALT lymphoma, and reviewed the histopathological findings. Correlations between the results of FISH analysis, the histopathological features and the clinical data were also analyzed.ResultsAmong the 34 cases, FISH analysis revealed t(14;18)(q32;q21) in one case, trisomy 3 in 21 cases (62%), and trisomy 18 in 16 cases (47%). The cases with trisomy 18 had significantly more prominent lymphoepithelial lesions (LELs) and less nodularity in the tumors. In regard to the clinical correlations, tumors with trisomy 18 were observed predominantly in females and younger patients; also, in the majority of the cases, the tumor was of conjunctival origin. All the cases with recurrence showed trisomy 18 in the tumor.ConclusionPrimary ocular adnexal MALT lymphoma is a significantly heterogeneous entity. Cases with trisomy 18 may have unique clinicopathological features.