title: Ocular adnexal marginal zone B cell lymphoma: a clinical and pathologic study of 23 cases.
abstract: To better characterize ocular adnexal marginal zone lymphoma of mucosa-associated lymphoid tissue (MZL-MALT), we analyzed the clinical and pathologic features of 23 patients (11 men, 12 women, median age 66 years). The tumor was confined to one ocular structure in 18 cases (conjunctiva, n=8; orbit, n=8; or lacrimal gland, n=2). Concurrent extraorbital disease was detected by the staging procedure in five patients, and preferentially involved other MALT sites. Histogenetic B cell marker studies, available in 13 cases, showed an early post-germinal center (GC) phenotype (BCL-6(-)/IRF4(+)/CD138(-)) (n=5) or a late post-GC phenotype (BCL-6(-)/IRF4(+)/CD138(+)) (n=8), which could be helpful for discrimination from other types of small-B cell lymphoma. BCL10 was positive in 12 of 13 patients tested, with nuclear (n=4) or cytoplasmic (n=8) immunoreactivity. These staining patterns ruled out t(1;14)(p22;q32) translocation. T(11;18)(q21;q21), another MZL-MALT-specific translocation, was detected by reverse transcriptase polymerase chain reaction in four of 15 patients tested. Clinical outcome was excellent but the overall relapse rate was 26.1% with a median follow-up of 39 months (range 6-132 months). Regardless of the disease stage at diagnosis, combined chemotherapy and radiotherapy seemed to be more effective than chemotherapy alone in ocular adnexal MZL-MALT, as persistent complete remission was achieved in nine patients receiving combination therapy, while six of 14 patients treated with chemotherapy alone relapsed.
authors.affiliation: Department of Pathology, Service d'Anatomie Pathologique, Hôpital Pitié-Salpêtrière, Boulevard de l'Hôpital, Paris, France. frederic.charlotte@psl.ap-hop-paris.fr
authors.forename: Frédéric
authors.initials: F
authors.lastname: Charlotte
authors.forename: Kamal
authors.initials: K
authors.lastname: Doghmi
authors.forename: Nathalie
authors.initials: N
authors.lastname: Cassoux
authors.forename: Hongtao
authors.initials: H
authors.lastname: Ye
authors.forename: Ming-Qing
authors.initials: MQ
authors.lastname: Du
authors.forename: Michèle
authors.initials: M
authors.lastname: Kujas
authors.forename: Annette
authors.initials: A
authors.lastname: Lesot
authors.forename: George
authors.initials: G
authors.lastname: Mansour
authors.forename: Phuc
authors.initials: P
authors.lastname: Lehoang
authors.forename: Nicole
authors.initials: N
authors.lastname: Vignot
authors.forename: Frédérique
authors.initials: F
authors.lastname: Capron
authors.forename: Véronique
authors.initials: V
authors.lastname: Leblond
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D014408:Biomarkers, Tumor / Q000032:analysis; D002467:Cell Nucleus / Q000737:chemistry / Q000473:pathology; D003131:Combined Modality Therapy; D003593:Cytoplasm / Q000737:chemistry / Q000473:pathology; D003937:Diagnosis, Differential; D005134:Eye Neoplasms / Q000737:chemistry / Q000235:genetics / Q000473:pathology* / Q000628:therapy; D005260:Female; D019085:Fluorescent Antibody Technique, Direct; D018858:Germinal Center / Q000737:chemistry / Q000473:pathology; D006801:Humans; D018442:Lymphoma, B-Cell, Marginal Zone / Q000737:chemistry / Q000235:genetics / Q000473:pathology* / Q000628:therapy; D008297:Male; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local; D009918:Orbital Neoplasms / Q000737:chemistry / Q000235:genetics / Q000473:pathology* / Q000628:therapy; D012074:Remission Induction; D020133:Reverse Transcriptase Polymerase Chain Reaction; D014178:Translocation, Genetic
chemical_list: D014408:Biomarkers, Tumor