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Ocular adnexal marginal zone B cell lymphoma: a clinical and pathologic study of 23 cases

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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.

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Authors and Affiliations

  1. Department of Pathology, Service d’Anatomie Pathologique (Pr F. Capron), Hôpital Pitié-Salpêtrière, Boulevard de l’hôpital, 75013, Paris, France

    Frédéric Charlotte, Michèle Kujas, Annette Lesot, George Mansour, Nicole Vignot & Frédérique Capron

  2. Department of Ophthalmology, Pitié-Salpêtrière Hospital, AP-HP, Paris, France

    Nathalie Cassoux & Phuc Lehoang

  3. Department of Hematology, Pitié-Salpêtrière Hospital, AP-HP, Paris, France

    Kamal Doghmi & Véronique Leblond

  4. Department of Histopathology, University College of London, London, UK

    Hongtao Ye & Ming-Qing Du

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  1. Frédéric Charlotte
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  2. Kamal Doghmi
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  3. Nathalie Cassoux
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  4. Hongtao Ye
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Correspondence to Frédéric Charlotte.

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Charlotte, F., Doghmi, K., Cassoux, N. et al. Ocular adnexal marginal zone B cell lymphoma: a clinical and pathologic study of 23 cases. Virchows Arch 448, 506–516 (2006). https://doi.org/10.1007/s00428-005-0122-0

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