Clinical and Experimental Medicine

, Volume 18, Issue 2, pp 151–163 | Cite as

Ocular adnexal marginal zone lymphoma of mucosa-associated lymphoid tissue

  • Dimitrios Kalogeropoulos
  • Alexandra Papoudou-Bai
  • Panagiotis Kanavaros
  • Chris Kalogeropoulos
Review Article


Ocular adnexal lymphomas are a group of heterogeneous neoplasms representing approximately 1–2% of non-Hodgkin lymphomas and 8% of extranodal lymphomas. The incidence of primary ocular adnexal lymphoid tumors has raised over the last decades, and this could be probably attributed to the more sophisticated diagnostic techniques. Due to the wide spectrum of clinical manifestations, ocular tissue biopsy is important in order to set a precise diagnosis based on histological, immunophenotypical and, in some cases, molecular findings. The most common subtype, which may account for up to 80% of primary ocular adnexal lymphomas, is extranodal marginal zone lymphoma (EMZL) of mucosa-associated lymphoid tissue. This lymphoma is usually asymptomatic in the early phase of the disease causing a delay in the final diagnosis and prompt therapy. The pathogenesis of a proportion of these tumors has been linked to chronic inflammatory stimulation from specific infectious factors (e.g., Chlamydia psittaci) or to autoimmunity. The further improvement in diagnostic methods and the further understanding of the pathogenesis of ocular adnexal EMZL may contribute to the establishment of a more successful multidisciplinary therapeutic planning.


Ocular adnexal lymphomas Immunohistochemistry Extranodal marginal zone lymphoma Mucosa-associated lymphoid tissue Chronic inflammatory stimulation 


Compliance with ethical standards

Conflicts of interest

All authors declare that there is no conflict of interest.

Ethical approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.

Informed consent

Informed consent was obtained from all individual participants included in the study.


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Copyright information

© Springer International Publishing AG 2017

Authors and Affiliations

  1. 1.Department of Ophthalmology, Faculty of Medicine, School of Health SciencesUniversity of IoanninaIoanninaGreece
  2. 2.Department of Pathology, Faculty of Medicine, School of Health SciencesUniversity of IoanninaIoanninaGreece
  3. 3.Department of Anatomy-Histology-Embryology, Faculty of Medicine, School of Health SciencesUniversity of IoanninaIoanninaGreece

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