Virchows Archiv

, Volume 448, Issue 4, pp 506–516

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

  • Frédéric Charlotte
  • Kamal Doghmi
  • Nathalie Cassoux
  • Hongtao Ye
  • Ming-Qing Du
  • Michèle Kujas
  • Annette Lesot
  • George Mansour
  • Phuc Lehoang
  • Nicole Vignot
  • Frédérique Capron
  • Véronique Leblond
Original Article


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.


MALT Lymphoma 


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Copyright information

© Springer-Verlag 2005

Authors and Affiliations

  • Frédéric Charlotte
    • 1
  • Kamal Doghmi
    • 3
  • Nathalie Cassoux
    • 2
  • Hongtao Ye
    • 4
  • Ming-Qing Du
    • 4
  • Michèle Kujas
    • 1
  • Annette Lesot
    • 1
  • George Mansour
    • 1
  • Phuc Lehoang
    • 2
  • Nicole Vignot
    • 1
  • Frédérique Capron
    • 1
  • Véronique Leblond
    • 3
  1. 1.Department of Pathology, Service d’Anatomie Pathologique (Pr F. Capron)Hôpital Pitié-SalpêtrièreParisFrance
  2. 2.Department of OphthalmologyPitié-Salpêtrière Hospital, AP-HPParisFrance
  3. 3.Department of HematologyPitié-Salpêtrière Hospital, AP-HPParisFrance
  4. 4.Department of HistopathologyUniversity College of LondonLondonUK

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