Extranodal Marginal Zone Lymphoma
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 unc
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Extranodal Marginal Zone Lymphoma Umberto Ricardi, Andrea Riccardo Filippi, Cristina Piva, and Mario Levis
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.
Introduction Indolent nonfollicular B-cell lymphoma (INFBCL) subtype has been classified in the World Health Organization (WHO) system U. Ricardi (*) • A.R. Filippi • C. Piva • M. Levis Department of Oncology, University of Torino, Via Genova 3, Torino 10125, Italy e-mail: [email protected]; [email protected]; [email protected]; [email protected]
among the mature B-cell neoplasms and includes nodal marginal zone lymphoma (NMZL), splenic marginal zone lymphoma (SMZL), extranodal marginal zone lymphoma (EN-MZL) of mucosaassociated lymphoid tissue (MALT), lymphoplasmacytic lymphoma (LPL), and small lymphocytic lymphoma (SLL) [79]. Marginal zone MALT lymphoma is the most frequent subtype among all marginal zone lymphomas (MZLs), accounting for 7 % of non-Hodgkin lymphoma (NHL); NMZL and SMZL represent only 2 % and 1 % of NHL, respectively [3, 54].
© Springer International Publishing Switzerland 2017 B.S. Dabaja, A.K. Ng (eds.), Radiation Therapy in Hematologic Malignancies, DOI 10.1007/978-3-319-42615-0_4
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56 Fig. 4.1 Frequency rates of extranodal marginal zone lymphomas (MZL) according to site (excluding the skin and spleen), SEER data (Khalil et al. [40])
Extranodal MZL Gastric 14.5 %
Ocular/adnexal
13.0 % 10.0 %
Head & Neck Bowel
9.5 %
38.5 %
Lung 7.5 %
3.0 %
4.0 % Breast Thyroid Others
MALT lymphoma is a distinct B-cell lymphoma that develops in extranodal sites. MALT lymphoma differs from its splenic and nodal counterparts because it arises in organs (e.g., stomach, ocular adnexal, and lung) that normally lack lymphoid tissue but accumulate B cells in response to chronic infections or autoimmune processes. The most common extranodal MALT lymphoma involves the gastrointestinal tract (about 50 % of MALT lymphomas), followed by non-gastric sites such as t
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