Ocular adnexal MALT lymphoma: An intriguing model for antigen-driven lymphomagenesis and microbial-targeted therapy

Andres J M Ferreri, R. Dolcetti, M. Q. Du, C. Doglioni, A. Giordano Resti, L. S. Politi, C. De Conciliis, J. Radford, F. Bertoni, E. Zucca, F. Cavalli, M. Ponzoni

Research output: Contribution to journalArticlepeer-review

Abstract

Non-Hodgkin's lymphomas constitute one half of malignancies arising in the orbit and the ocular adnexae. Mucosa-associated lymphoid tissue (MALT)-type lymphoma is the most common histological category in this anatomic region. The incidence of ocular adnexal lymphoma of mucosa-associated lymphoid tissue-type (OAML) is increasing and recent studies offered new relevant insights in molecular, pathogenetic and therapeutic issues on these neoplasms. A pathogenetic model of antigen-driven lymphoproliferation similar to that reported for Helicobacter pylori-related gastric MALT lymphomas has been hypothesized for OAML. This notion is supported by the association between OAML and Chlamydophila psittaci infection, an association that is of likely pathogenetic relevance and may influence both the biological behavior and the therapeutic management of these neoplasms. However, this association displays evident geographical variability indicating that other etiopathogenic agents could be involved. These recent acquisitions coupled with the occurrence of chromosomal translocations and other genetic alterations, as well as additional risk factors like autoimmune disorders have contributed to render OAML an exciting challenge for a broad group of physicians and scientists. OAML is an indolent and rarely lethal malignancy that, in selected patients, can be managed with observation alone. Lymphomatous lesions are frequently responsible for symptoms affecting patient's quality of life, requiring, therefore, immediate treatment. Several therapeutic strategies are available, often associated with relevant side-effects. However, the therapeutic choice in OAML is not supported by consolidated evidence due to the lack of prospective trials. In this review, we analyze the most relevant biological, molecular, pathological and clinical features of OAML and propose some therapeutic guidelines for patients affected by this malignancy.

Original languageEnglish
Pages (from-to)835-846
Number of pages12
JournalAnnals of Oncology
Volume19
Issue number5
DOIs
Publication statusPublished - May 2008

Keywords

  • Chlamydia
  • Extranodal lymphomas
  • Interferon
  • MALT
  • Ocular adnexae
  • Rituximab

ASJC Scopus subject areas

  • Oncology
  • Cancer Research

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