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Extranodal Marginal Zone Lymphoma

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Abstract

Extranodal marginal zone lymphomas (EN-MZLs) comprise about 25 % of all non-Hodgkin lymphomas (NHL). The wide range of presentations of EN-MZL, involving virtually any organ, poses several challenges. The heterogeneity of the disease also explains the uncertainty and lack of consistency in radiotherapy approaches between centers and clinicians. EN-MZL often presents with localized/organ-confined disease, and RT is generally the primary curative modality. High local control rates can be achieved with relatively modest radiation doses, and local control often translates to cure.

In this chapter, we shortly present two clinical cases of the most common EN-MZL subtypes (gastric and ocular adnexa MZL), discussing diagnosis, staging, and therapeutic strategies, with a special focus on the role of radiotherapy. Current recommendations in terms of optimal dose, volumes, and techniques are also presented. Few tables summarize the results of the largest series reporting on the outcome following radiotherapy, and figures illustrate imaging features as well as contouring and planning examples.

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  1. Department of Oncology, University of Torino, Via Genova 3, Torino, 10125, Italy

    Umberto Ricardi, Andrea Riccardo Filippi, Cristina Piva & Mario Levis

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  2. Andrea Riccardo Filippi
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  1. University of Texas, MD Anderson Cancer Center, Houston, Texas, USA

    Bouthaina Shbib Dabaja

  2. Dana-Farber Cancer Institute, Harvard University Medical School, Boston, Massachusetts, USA

    Andrea K. Ng

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Ricardi, U., Filippi, A.R., Piva, C., Levis, M. (2017). Extranodal Marginal Zone Lymphoma. In: Dabaja, B., Ng, A. (eds) Radiation Therapy in Hematologic Malignancies . Springer, Cham. https://doi.org/10.1007/978-3-319-42615-0_4

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