Annals of Oncology Advance Access originally published online on November 6, 2007
Annals of Oncology 2008 19(5):835-846; doi:10.1093/annonc/mdm513
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Ocular adnexal MALT lymphoma: an intriguing model for antigen-driven lymphomagenesis and microbial-targeted therapy
1 Unit of Lymphoid Malignancies
2 Medical Oncology Unit, Department of Oncology, San Raffaele Scientific Institute, Milan
3 Cancer Bio-Immunotherapy Unit, Department of Medical Oncology, Centro di Riferimento Oncologico, IRCCS National Cancer Institute, Aviano, Italy
4 Division of Molecular Histopathology, Department of Pathology, University of Cambridge, Addenbrooke's Hospital, Cambridge, UK
5 Pathology Unit
6 Ophthalmology Unit
7 Neuroradiology Unit, San Raffaele Scientific Institute, Milan
8 Ophthalmology Unit, Ospedale San Giuseppe, Milan, Italy
9 Cancer Research UK, Department of Medical Oncology, Christie Hospital NHS Trust, Manchester, UK
10 Oncology Institute of Southern Switzerland, Ospedale San Giovanni, Bellinzona, Switzerland
* Correspondence to: A. J. M. Ferreri MD, Medical Oncology Unit, Department of Oncology, San Raffaele Scientific Institute, Via Olgettina 60, 20132, Milan, Italy. Tel: 0039-02-26437649; Fax: 0039-02-26437625; E-mail: andres.ferreri{at}hsr.it
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.
Key words: chlamydia, extranodal lymphomas, interferon, MALT, ocular adnexae, rituximab
Received for publication July 11, 2007. Revision received September 26, 2007. Accepted for publication October 5, 2007.
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