Abstract
Marginal zone lymphoma (MZL) is an indolent disease with a favorable prognostic outcome. MZLs account for 5–17% of all non-Hodgkin’s lymphomas (NHL) in adults. During the course of past years, the incidence of marginal zone lymphomas increased, caused by changes in diagnostic practice. MZLs represent a group of lymphomas which originate from memory B lymphocytes being present in the “marginal zone” of the secondary lymphoid follicles. Depending on the site of involvement of MZL cells, the International Lymphoma Study group differentiates MZL into three different subtypes: nodal MZL (10%), splenic MZL (20%), and the most frequent form of extranodal MZL (70%). Extranodal MZLs (ENMZLs) occur at any site of the body, with the stomach being the most common extranodal site, followed by ocular, bowel, lung, and salivary glands’ infiltration. They are generally associated with chronic antigenic stimulation as a result of infection or autoimmune disease. The clinical symptoms of ENMZL vary strongly depending on the different sites of lymphoma involvement. Most patients present with early-stage I or II ENMZL. Extranodal MZLs are treated with various modalities including surgical therapy, radiation therapy, and systemic therapy. Radiotherapy plays an important part in contributing to preservation of native organ function. Treatment with curative intent achieves high disease-free and overall survival rates and decreases the frequency of relapses.
This is a preview of subscription content, log in via an institution to check access.
References
Ardeshna KM, Smith P, Norton A, et al. Long-term effect of a watch and wait policy versus immediate systemic treatment for asymptomatic advanced-stage non-Hodgkin lymphoma: a randomised controlled trial. Lancet Lond Engl. 2003;362:516–22.
Avilés A, Delgado S, Huerta-Guzmán J. Marginal zone B cell lymphoma of the parotid glands: results of a randomised trial comparing radiotherapy to combined therapy. Eur J Cancer B Oral Oncol. 1996;32B:420–2.
Brice P, Bastion Y, Lepage E, et al. Comparison in low-tumor-burden follicular lymphomas between an initial no-treatment policy, prednimustine, or interferon alfa: a randomized study from the Groupe d’Etude des Lymphomes Folliculaires. Groupe d’Etude des Lymphomes de l’Adulte. J Clin Oncol Off J Am Soc Clin Oncol. 1997;15:1110–7. https://doi.org/10.1200/JCO.1997.15.3.1110.
Cefaro GA, Valentini V, Perez CA. Delineating organs at risk in radiation therapy. Milan: Springer; 2013.
Conconi A, Martinelli G, Thiéblemont C, et al. Clinical activity of rituximab in extranodal marginal zone B-cell lymphoma of MALT type. Blood. 2003;102:2741–5. https://doi.org/10.1182/blood-2002-11-3496.
Decaudin D. Ocular adnexal lymphoma: a review of clinicopathologic features and treatment options. Blood. 2006;108:1451–60. https://doi.org/10.1182/blood-2006-02-005017.
Della Biancia C, Hunt M, Furhang E, et al. Radiation treatment planning techniques for lymphoma of the stomach. Int J Radiat Oncol. 2005;62:745–51. https://doi.org/10.1016/j.ijrobp.2004.10.025.
Dreyling M, Thieblemont C, Gallamini A, et al. ESMO consensus conferences: guidelines on malignant lymphoma. Part 2: marginal zone lymphoma, mantle cell lymphoma, peripheral T-cell lymphoma. Ann Oncol Off J Eur Soc Med Oncol. 2013;24:857–77. https://doi.org/10.1093/annonc/mds643.
Ferraro P, Trastek VF, Adlakha H, et al. Primary non-Hodgkin’s lymphoma of the lung. Ann Thorac Surg. 2000;69:993–7. https://doi.org/10.1016/S0003-4975(99)01535-0.
Ferreri AJM, Govi S, Pasini E, et al. Chlamydophila psittaci eradication with doxycycline as first-line targeted therapy for ocular adnexae lymphoma: final results of an international phase II trial. J Clin Oncol Off J Am Soc Clin Oncol. 2012;30:2988–94. https://doi.org/10.1200/JCO.2011.41.4466.
Fischbach W. Long term outcome of patients with gastric marginal zone B cell lymphoma of mucosa associated lymphoid tissue (MALT) following exclusive Helicobacter pylori eradication therapy: experience from a large prospective series. Gut. 2004;53:34–7. https://doi.org/10.1136/gut.53.1.34.
Fischbach W, Goebeler-Kolve M-E, Greiner A. Diagnostic accuracy of EUS in the local staging of primary gastric lymphoma: results of a prospective, multicenter study comparing EUS with histopathologic stage. Gastrointest Endosc. 2002;56:696–700. https://doi.org/10.1067/mge.2002.128915.
Fischbach W, Goebeler ME, Ruskone-Fourmestraux A, et al. Most patients with minimal histological residuals of gastric MALT lymphoma after successful eradication of Helicobacter pylori can be managed safely by a watch and wait strategy: experience from a large international series. Gut. 2007;56:1685–7. https://doi.org/10.1136/gut.2006.096420.
Fleming ID, Mitchell S, Dilawari RA. The role of surgery in the management of gastric lymphoma. Cancer. 1982;49:1135–41.
Goda JS, Gospodarowicz M, Pintilie M, et al. Long-term outcome in localized extranodal mucosa-associated lymphoid tissue lymphomas treated with radiotherapy. Cancer. 2010;116:3815–24. https://doi.org/10.1002/cncr.25226.
Hitchcock S, Ng AK, Fisher DC, et al. Treatment outcome of mucosa-associated lymphoid tissue/marginal zone non-Hodgkin’s lymphoma. Int J Radiat Oncol. 2002;52:1058–66. https://doi.org/10.1016/S0360-3016(01)02714-6.
Isaacson PG, Chott A, Nakamura S, Muller-Hermelink HK. Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma). In: WHO classification of Tumours of Haematopoietic and lymphoid tissues. Lyon: IARC Press; 2008. p. 214–7.
Juweid ME, Stroobants S, Hoekstra OS, et al. Use of positron emission tomography for response assessment of lymphoma: consensus of the Imaging Subcommittee of International Harmonization Project in Lymphoma. J Clin Oncol Off J Am Soc Clin Oncol. 2007;25:571–8. https://doi.org/10.1200/JCO.2006.08.2305.
Kamdar MK, Smith SM. Extranodal marginal zone lymphoma: No longer Just a sidekick. J Clin Oncol Off J Am Soc Clin Oncol. 2017;35:1872. https://doi.org/10.1200/JCO.2017.72.2835.
Kavanagh BD, Pan CC, Dawson LA, et al. Radiation dose–volume effects in the stomach and small bowel. Int J Radiat Oncol. 2010;76:S101–7. https://doi.org/10.1016/j.ijrobp.2009.05.071.
Khalil MO, Morton LM, Devesa SS, et al. Incidence of marginal zone lymphoma in the United States, 2001-2009 with a focus on primary anatomic site. Br J Haematol. 2014;165:67–77. https://doi.org/10.1111/bjh.12730.
Koch P, del Valle F, Berdel WE, et al. Primary gastrointestinal non-Hodgkin’s lymphoma: I. Anatomic and histologic distribution, clinical features, and survival data of 371 patients registered in the German multicenter study GIT NHL 01/92. J Clin Oncol Off J Am Soc Clin Oncol. 2001a;19:3861–73. https://doi.org/10.1200/JCO.2001.19.18.3861.
Koch P, del Valle F, Berdel WE, et al. Primary gastrointestinal non-Hodgkin’s lymphoma: II. Combined surgical and conservative or conservative management only in localized gastric lymphoma – results of the prospective German multicenter study GIT NHL 01/92. J Clin Oncol Off J Am Soc Clin Oncol. 2001b;19:3874–83. https://doi.org/10.1200/JCO.2001.19.18.3874.
Koch P, Probst A, Berdel WE, et al. Treatment results in localized primary gastric lymphoma: data of patients registered within the German multicenter study (GIT NHL 02/96). J Clin Oncol Off J Am Soc Clin Oncol. 2005;23:7050–9. https://doi.org/10.1200/JCO.2005.04.031.
Kodera Y, Yamamura Y, Nakamura S, et al. The role of radical gastrectomy with systematic lymphadenectomy for the diagnosis and treatment of primary gastric lymphoma. Ann Surg. 1998;227:45–50.
Le QT, Eulau SM, George TI, et al. Primary radiotherapy for localized orbital MALT lymphoma. Int J Radiat Oncol Biol Phys. 2002;52:657–63.
MacDermed D, Thurber L, George TI, et al. Extranodal nonorbital indolent lymphomas of the head and neck: relationship between tumor control and radiotherapy. Int J Radiat Oncol. 2004;59:788–95. https://doi.org/10.1016/j.ijrobp.2003.11.007.
Maor MH, North LB, Cabanillas FF, et al. Outcomes of high-dose unilateral kidney irradiation in patients with gastric lymphoma. Int J Radiat Oncol. 1998;41:647–50. https://doi.org/10.1016/S0360-3016(98)00083-2.
Martinelli G, Laszlo D, Ferreri AJM, et al. Clinical activity of rituximab in gastric marginal zone non-Hodgkin’s lymphoma resistant to or not eligible for anti-Helicobacter pylori therapy. J Clin Oncol Off J Am Soc Clin Oncol. 2005;23:1979–83. https://doi.org/10.1200/JCO.2005.08.128.
Morton LM, Wang SS, Devesa SS, et al. Lymphoma incidence patterns by WHO subtype in the United States, 1992-2001. Blood. 2006;107:265–76. https://doi.org/10.1182/blood-2005-06-2508.
Musshoff K. Clinical staging classification of non-Hodgkin’s lymphomas (author’s transl). Strahlentherapie. 1977;153:218–21.
Nakamura S, Sugiyama T, Matsumoto T, et al. Long-term clinical outcome of gastric MALT lymphoma after eradication of Helicobacter pylori: a multicentre cohort follow-up study of 420 patients in Japan. Gut. 2012;61:507–13. https://doi.org/10.1136/gutjnl-2011-300495.
Olszewski AJ, Castillo JJ. Survival of patients with marginal zone lymphoma: analysis of the Surveillance, Epidemiology, and End Results database. Cancer. 2013;119:629–38. https://doi.org/10.1002/cncr.27773.
Olszewski AJ, Desai A. Radiation therapy administration and survival in stage I/II extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue. Int J Radiat Oncol. 2014;88:642–9. https://doi.org/10.1016/j.ijrobp.2013.11.225.
Pinotti G, Zucca E, Roggero E, et al. Clinical features, treatment and outcome in a series of 93 patients with low-grade gastric MALT lymphoma. Leuk Lymphoma. 1997;26:527–37. https://doi.org/10.3109/10428199709050889.
Radaszkiewicz T, Dragosics B, Bauer P. Gastrointestinal malignant lymphomas of the mucosa-associated lymphoid tissue: factors relevant to prognosis. Gastroenterology. 1992;102:1628–38.
Ruskone-Fourmestraux A, Fischbach W, Aleman BMP, et al. EGILS consensus report. Gastric extranodal marginal zone B-cell lymphoma of MALT. Gut. 2011;60:747–58. https://doi.org/10.1136/gut.2010.224949.
Ruskoné-Fourmestraux A, Matysiak-Budnik T, Fabiani B, et al. Exclusive moderate-dose radiotherapy in gastric marginal zone B-cell MALT lymphoma: results of a prospective study with a long term follow-up. Radiother Oncol J Eur Soc Ther Radiol Oncol. 2015;117:178–82. https://doi.org/10.1016/j.radonc.2015.08.029.
Schechter NR, Portlock CS, Yahalom J. Treatment of mucosa-associated lymphoid tissue lymphoma of the stomach with radiation alone. J Clin Oncol. 1998;16:1916–21. https://doi.org/10.1200/JCO.1998.16.5.1916.
Senff NJ, Noordijk EM, Kim YH, et al. European Organization for Research and Treatment of cancer and International Society for Cutaneous Lymphoma consensus recommendations for the management of cutaneous B-cell lymphomas. Blood. 2008;112:1600–9. https://doi.org/10.1182/blood-2008-04-152850.
Stefanovic A, Lossos IS. Extranodal marginal zone lymphoma of the ocular adnexa. Blood. 2009;114:501–10. https://doi.org/10.1182/blood-2008-12-195453.
Swerdlow SH, Campo E, Harris NL, et al., editors. WHO classification of tumours of haematopoietic and lymphoid tissues: [... reflects the views of a working group that convened for an Editorial and Consensus Conference at the International Agency for Research on Cancer (IARC), Lyon, October 25–27, 2007]. 4th ed. Lyon: International Agency for Research on Cancer; 2008.
Teckie S, Qi S, Lovie S, et al. Long-term outcomes and patterns of relapse of early-stage Extranodal marginal zone lymphoma treated with radiation therapy with curative intent. Int J Radiat Oncol. 2015;92:130–7. https://doi.org/10.1016/j.ijrobp.2015.01.040.
Tsang RW, Gospodarowicz MK, Pintilie M, et al. Stage I and II MALT lymphoma: results of treatment with radiotherapy. Int J Radiat Oncol Biol Phys. 2001;50:1258–64.
Vrieling C, de Jong D, Boot H, et al. Long-term results of stomach-conserving therapy in gastric MALT lymphoma. Radiother Oncol. 2008;87:405–11. https://doi.org/10.1016/j.radonc.2008.02.012.
Wöhrer S, Kiesewetter B, Fischbach J, et al. Retrospective comparison of the effectiveness of various treatment modalities of extragastric MALT lymphoma: a single-center analysis. Ann Hematol. 2014;93:1287–95. https://doi.org/10.1007/s00277-014-2042-z.
Wotherspoon AC, Doglioni C, Diss TC, et al. Regression of primary low-grade B-cell gastric lymphoma of mucosa-associated lymphoid tissue type after eradication of Helicobacter pylori. Lancet Lond Engl. 1993;342:575–7.
Yahalom J, Illidge T, Specht L, et al. Modern radiation therapy for extranodal lymphomas: field and dose guidelines from the International Lymphoma Radiation Oncology Group. Int J Radiat Oncol Biol Phys. 2015;92:11–31. https://doi.org/10.1016/j.ijrobp.2015.01.009.
Zucca E, Bertoni F. The spectrum of MALT lymphoma at different sites: biological and therapeutic relevance. Blood. 2016;127:2082–92. https://doi.org/10.1182/blood-2015-12-624304.
Zucca E, Copie-Bergman C, Ricardi U, et al. Gastric marginal zone lymphoma of MALT type: ESMO clinical practice guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2013;24(Supplement 6):vi144–8. https://doi.org/10.1093/annonc/mdt343.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2017 Springer International Publishing AG
About this entry
Cite this entry
Reinartz, G., Weiglein, T., Dreyling, M., Oertel, M. (2017). Radiation Therapy in Marginal Zone B-Cell Lymphomas. In: Wenz, F. (eds) Radiation Oncology. Springer, Cham. https://doi.org/10.1007/978-3-319-52619-5_24-1
Download citation
DOI: https://doi.org/10.1007/978-3-319-52619-5_24-1
Received:
Accepted:
Published:
Publisher Name: Springer, Cham
Print ISBN: 978-3-319-52619-5
Online ISBN: 978-3-319-52619-5
eBook Packages: Springer Reference MedicineReference Module Medicine