Highlights & Basics
- Mucosa-associated lymphoid tissue (MALT) lymphoma is categorized as indolent or low-grade B-cell lymphoma; however, high-grade histologic transformation can occur.
- Median age at presentation is approximately 60 years.
- The stomach is the most frequently involved organ, and in most cases (90%), there is a strong association between gastric MALT lymphoma and chronic Helicobacter pylori infection.
- Other sites that may be involved include the ocular adnexa, lung, parotid gland, skin, intestinal tract, and thyroid. May also occur (rarely) in breasts, dura, and genitourinary tract. Autoimmune diseases have been linked to nongastric MALT lymphoma.
- Diagnosis is based on history, physical exam, radiologic imaging studies, histopathologic and immunohistochemical evaluation of the biopsy specimen, and molecular laboratory techniques.
Quick Reference
History & Exam
Key Factors
Other Factors
Diagnostics Tests
Treatment Options
Definition
Epidemiology
Etiology
Pathophysiology
Images
Infiltrate of lymphoid cells in the lung, confirming their B-cell origin (CD20 staining, ×200)
MALT lymphoma of the lung: residual respiratory epithelium has been distorted by infiltrating lymphocytes; the lymphoepithelial lesion (cytokeratin staining, ×200)
MALT lymphoma of the lung: parts of this tumor demonstrate striking plasmacytic differentiation (CD138 staining, ×200)
Gastric MALT lymphoma: infiltration of the gastric epithelium by neoplastic B-lymphocytes (CD20 staining, ×200)
MALT lymphoma of the lung: lung parenchyma has been replaced by a neoplastic infiltrate of small lymphocytes; a follicle surrounded by neoplastic marginal zone cells can be recognized in the center of the image (hematoxylin and eosin [H&E] staining, ×200)
Gastric MALT lymphoma: normal gastric epithelium distorted by a neoplastic infiltrate of lymphocytes extending into the superficial gastric epithelium (hematoxylin and eosin [H&E] staining, × 200)
Citations
National Comprehensive Cancer Network. NCCN clinical practice guidelines in oncology: B-cell lymphomas [internet publication].[Full Text]
Zucca E, Arcaini L, Buske C, et al. Marginal zone lymphomas: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2020 Jan;31(1):17-29.[Abstract][Full Text]
1. Jaffe E S, Harris N L, Stein H, et al. Pathology and genetics of tumours of haematopoietic and lymphoid tissue. Lyon: International Agency for Research on Cancer; 2001.
2. Campo E, Jaffe ES, Cook JR, et al. The International Consensus Classification of Mature Lymphoid Neoplasms: a report from the Clinical Advisory Committee. Blood. 2022 Sep 15;140(11):1229-53.[Abstract][Full Text]
3. Jaffe ES, Harris NL, Stein H, et al, eds. World Health Organization classification of tumors. Pathology and genetics: tumors of haemopoietic and lymphoid tissues. Lyon, France: IARC Press; 2008.
4. Alaggio R, Amador C, Anagnostopoulos I, et al. The 5th edition of the World Health Organization Classification of haematolymphoid tumours: lymphoid neoplasms. Leukemia. 2022 Jul;36(7):1720-48.[Abstract][Full Text]
5. Harris NL, Jaffe ES, Stein H, et al. A revised European-American classification of lymphoid neoplasms: a proposal from the International Lymphoma Study Group. Blood. 1994;84:1361-1392.[Abstract][Full Text]
6. Zucca E, Conconi A, Pedrinis E, et al. Nongastric marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue. Blood. 2003 Apr 1;101(7):2489-95.[Abstract][Full Text]
7. Cohen SM, Petryk M, Varma M, et al. Non-Hodgkin's lymphoma of mucosa-associated lymphoid tissue. Oncologist. 2006;11:1100-17.[Abstract]
8. The Non-Hodgkin's Lymphoma Classification Project. A clinical evaluation of the International Lymphoma Study Group classification of non-Hodgkin's lymphoma. Blood. 1997;89:3909-3918.[Abstract][Full Text]
9. Di Rocco A, Petrucci L, Assanto GM, et al. Extranodal marginal zone lymphoma: pathogenesis, diagnosis and treatment. Cancers (Basel). 2022 Mar 29;14(7):1742[Abstract][Full Text]
10. Raderer M, Kiesewetter B, Ferreri AJ. Clinicopathologic characteristics and treatment of marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma). CA Cancer J Clin. 2016 Mar-Apr;66(2):153-71.[Abstract][Full Text]
11. Kiesewetter B, Lamm W, Dolak W, et al. Transformed mucosa-associated lymphoid tissue lymphomas: a single institution retrospective study including polymerase chain reaction-based clonality analysis. Br J Haematol. 2019 Aug;186(3):448-59.[Abstract][Full Text]
12. Doglioni C, Wotherspoon AC, Moschini A, et al. High incidence of primary gastric lymphoma in northeastern Italy. Lancet. 1992;339:834-835.[Abstract]
13. Pinkel D. Differentiating juvenile myelomonocytic leukemia from infectious disease. Blood. 1998;91:365-367.[Abstract][Full Text]
14. Price SK. Immunoproliferative small intestinal disease: a study of 13 cases with alpha heavy-chain disease. Histopathology. 1990;17:7-17.[Abstract]
15. Zucca E, Conconi A, Roggero E, et al. Non-gastric MALT lymphomas: a survey of 369 European patients. The International Extranodal Lymphoma Study Group. Ann Oncol. 2000;11:99.
16. Kahl B, Yang D. Marginal zone lymphomas: management of nodal, splenic, and MALT NHL. Haematology Am Soc Hematol Educ Program. 2008:359-64.[Abstract][Full Text]
17. Diss TC, Wotherspoon AC, Speight P, et al. B-cell monoclonality, Epstein Barr virus and t(14;18) in myoepithelial sialadenitis and low-grade B-cell MALT lymphoma of the parotid gland. Am J Surg Pathol. 1995;19:531-536.[Abstract]
18. Ansell SM, Grant CS, Habermann TM. Primary thyroid lymphoma. Semin Oncol. 1999;26:316-323.[Abstract]
19. Kassan SS, Thomas TL, Moutsopoulos HM, et al. Increased risk of lymphoma in sicca syndrome. Ann Intern Med. 1978;89:888-892.[Abstract]
20. Talan N, Sokoloff L, Barth WF. Extrasalivary lymphoid abnormalities in Sjogren's syndrome (reticulum cell sarcoma, "pseudolymphoma," macroglobulinemia). Am J Med. 1967;43:50-65.[Abstract]
21. Cerroni L, Zochling N, Putz B, et al. Infection by Borrelia burgdorferi and cutaneous B-cell lymphoma. J Cutan Pathol. 1997;24:457-461.[Abstract]
22. Ferreri AJ, Ponzoni M, Guidoboni M, et al. Bacteria-eradicating therapy with doxycycline in ocular adnexal MALT lymphoma: a multicenter prospective trial. J Natl Cancer Inst. 2006;98:1375-1382.[Abstract][Full Text]
23. Lecuit M, Abachin E, Martin A, et al. Immunoproliferative small intestinal disease associated with Campylobacter jejuni. N Engl J Med. 2004;350:239-248.[Abstract][Full Text]
24. Luppi M, Longo G, Ferrari MG, et al. Additional neoplasms and HCV infection in low-grade lymphoma of MALT type. Br J Haematol. 1996;94:373-375.[Abstract]
25. Ascoli V, Lo Coco F, Artini M, et al. Extranodal lymphomas associated with hepatitis C virus infection. Am J Clin Pathol. 1998;109:600-609.[Abstract]
26. Zucca E, Roggero E, Maggi-Solca N, et al. Prevalence of Helicobacter pylori and hepatitis C virus infections among non-Hodgkin's lymphoma patients in Southern Switzerland. Haematologica. 2000;85:147-153.[Abstract]
27. Girard T, Luquet-Besson I, Baran-Marszak F, et al. HIV+ MALT lymphoma remission induced by highly active antiretroviral therapy alone. Eur J Haematol. 2005;74:70-72. [Abstract]
28. Isaacson PG. Gastric MALT lymphoma: from concept to cure. Ann Oncol. 1999;10:637-645.[Abstract][Full Text]
29. de Jong D, Boot H, van Heerde P, et al. Histological grading in gastric lymphoma: pretreatment criteria and clinical relevance. Gastroenterology. 1997;112:1466-1474.[Abstract]
30. Liu H, Ye H, Ruskone-Fourmestraux A, et al. T(11;18) is a marker for all stage gastric MALT lymphomas that will not respond to H. pylori eradication. Gastroenterology. 2002;122:1286-1294.[Abstract]
31. Ye H, Liu H, Raderer M, et al. High incidence of t(11;18)(q21;q21) in Helicobacter pylori-negative gastric MALT lymphoma. Blood. 2003;101:2547-2550.[Abstract][Full Text]
32. Liu H, Ye H, Dogan A, et al. T(11;18)(q21;q21) is associated with advanced mucosa-associated lymphoid tissue lymphoma that expresses nuclear BCL10. Blood. 2001;98:1182-1187.[Abstract]
33. Starostik P, Patzner J, Greiner A, et al. Gastric marginal zone B-cell lymphomas of MALT type develop along 2 distinct pathogenetic pathways. Blood. 2002;99:3-9.[Abstract][Full Text]
34. National Comprehensive Cancer Network. NCCN clinical practice guidelines in oncology: B-cell lymphomas [internet publication].[Full Text]
35. Zucca E, Arcaini L, Buske C, et al. Marginal zone lymphomas: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2020 Jan;31(1):17-29.[Abstract][Full Text]
36. Molina TJ, Lin P, Swerdlow SH, et al. Marginal zone lymphomas with plasmacytic differentiation and related disorders. Am J Clin Pathol. 2011 Aug;136(2):211-25.[Abstract][Full Text]
37. Nakamura S, Aoyagi K, Furuse M, et al. B-cell monoclonality precedes the development of gastric MALT lymphoma in Helicobacter pylori-associated chronic gastritis. Am J Pathol. 1998;152:1271-1279.[Abstract][Full Text]
38. Zucca E, Bertoni F, Roggero E, et al. Molecular analysis of the progression from Helicobacter pylori-associated chronic gastritis to mucosa-associated lymphoid-tissue lymphoma of the stomach. N Engl J Med. 1998;338:804-810.[Abstract][Full Text]
39. Cavalli F, Isaacson PG, Gascoyne RD, et al. MALT lymphomas. Haematology Am Soc Hematol Educ Program. 2001;241-258.[Abstract][Full Text]
40. Kahl BS. Update: gastric MALT lymphoma. Curr Opin Oncol. 2003;15:347-352.[Abstract]
41. Streubel B, Huber D, Wohrer S, et al. Frequency of chromosomal aberrations involving MALT1 in mucosa-associated lymphoid tissue lymphoma in patients with Sjogren's syndrome. Clin. Cancer Res. 2004;10:476-480.[Abstract][Full Text]
42. Ramstein ED, Dogan A, Einerson RR, et al. The incidence and anatomic site specificity of chromosomal translocations in primary extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) in North America. Am J Surg Pathol. 2006;30:1546-1553.[Abstract]
43. Perry C, Herishanu Y, Metzer U, et al. Diagnostic accuracy of PET/CT in patients with extranodal marginal zone MALT lymphoma. Eur J Haematol. 2007;79:205-209.[Abstract]
44. Almuhaideb A, Papathanasiou N, Bomanji J. 18F-FDG PET/CT imaging in oncology. Ann Saudi Med. 2011 Jan-Feb;31(1):3-13.[Abstract][Full Text]
45. Carrillo-Cruz E, Marín-Oyaga VA, de la Cruz Vicente F, et al. Role of 18F-FDG-PET/CT in the management of marginal zone B cell lymphoma. Hematol Oncol. 2015 Dec;33(4):151-8.[Abstract][Full Text]
46. Albano D, Giubbini R, Bertagna F. 18F-FDG PET/CT and primary hepatic MALT: a case series. Abdom Radiol (NY). 2016 Oct;41(10):1956-9.[Abstract][Full Text]
47. Nakamura S, Yao T, Aoyagi K, et al. Helicobacter pylori and primary gastric lymphoma: a histopathologic and immunohistochemical analysis of 237 patients. Cancer. 1997 Jan 1;79(1):3-11.[Abstract]
48. Fischbach W. Endosonographic diagnosis and treatment planning in gastrointestinal lymphoma. In: Dietrich CF, ed. Endoscopic ultrasound: an introductory manual and atlas. New York, NY: Thieme; 2006:155-160.
49. Rohatiner A, d'Amore F, Coiffier B, et al. Report on a workshop convened to discuss the pathological and staging classifications of gastrointestinal tract lymphoma. Ann Oncol. 1994;5:397-400.[Abstract]
50. Ruskoné-Fourmestraux A, Dragosics B, Morgner A, et al. Paris staging system for primary gastrointestinal lymphomas. Gut. 2003 Jun;52(6):912-3.[Abstract][Full Text]
51. Fock KM, Katelaris P, Sugano K, et al; Second Asia-Pacific Conference. Second Asia-Pacific Consensus Guidelines for Helicobacter pylori infection. J Gastroenterol Hepatol. 2009;24:1587-1600.[Abstract]
52. Zullo A, Hassan C, Andriani A, et al. Eradication therapy for Helicobacter pylori in patients with gastric MALT lymphoma: a pooled data analysis. Am J Gastroenterol. 2009;104:1932-1937.[Abstract]
53. Levy M, Copie-Bergman C, Traulle C, et al. Conservative treatment of primary gastric low-grade B-cell lymphoma of mucosa-associated lymphoid tissue: predictive factors of response and outcome. Am J Gastroenterol. 2002;97:292-297.[Abstract]
54. Hancock BW, Qian W, Linch D, et al. Chlorambucil versus observation after anti-Helicobacter therapy in gastric MALT lymphomas: results of the international randomised LY03 trial. Br J Haematol. 2009;144:367-375.[Abstract][Full Text]
55. Schecter NR, Portlock CS, Yahalom J. Treatment of mucosa-associated lymphoid tissue lymphoma of the stomach with radiation alone. J Clin Oncol. 1998;16:1916-1921.[Abstract]
56. Yahalom J. MALT lymphomas: a radiation oncology viewpoint. Ann Hematol. 2001;80(suppl 3):B100S-B105S.[Abstract]
57. Tsang RW, Gospodarowicz MK, Pintilie M, et al. Localized mucosa-associated lymphoid tissue lymphoma treated with radiation therapy has excellent clinical outcome. J Clin Oncol. 2003;21:4157-4164.[Abstract]
58. Zullo A, Hassan C, Andriani A, et al. Treatment of low-grade gastric MALT-lymphoma unresponsive to Helicobacter pylori therapy: a pooled-data analysis. Med Oncol. 2010;27:291-295.[Abstract]
59. Martinelli G, Laszlo D, Ferreri AJ, 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. 2005 Mar 20;23(9):1979-83.[Abstract][Full Text]
60. Amiot A, Lévy M, Copie-Bergman C, et al. Rituximab, alkylating agents or combination therapy for gastric mucosa-associated lymphoid tissue lymphoma: a monocentric non-randomised observational study. Aliment Pharmacol Ther. 2014 Mar;39(6):619-28.[Abstract][Full Text]
61. Hammel P, Haioun C, Chaumette MT, et al. Efficacy of single-agent chemotherapy in low-grade B-cell mucosa-associated lymphoid tissue lymphoma with prominent gastric expression. J Clin Oncol. 1995;13:2524-2529.[Abstract]
62. Jager G, Neumeister P, Brezinschek R, et al. Treatment of extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue type with cladribine: a phase II study. J Clin Oncol. 2002;20:3872-3877.[Abstract]
63. Wohrer S, Drach J, Hejna M, et al. Treatment of extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) with mitoxantrone, chlorambucil and prednisone (MCP). Ann Oncol. 2003;14:1758-61.[Abstract][Full Text]
64. Zinzani PL, Stefoni V, Musuraca G, et al. Fludarabine-containing chemotherapy as frontline treatment of nongastrointestinal mucosa-associated lymphoid tissue lymphoma. Cancer. 2004;100:2190-2194.[Abstract][Full Text]
65. Raderer M, Wohrer S, Bartsch R, et al. Phase II study of oxaliplatin for treatment of patients with mucosa-associated lymphoid tissue lymphoma. J Clin Oncol. 2005;23:8442-8446.[Abstract]
66. Zucca E, Conconi A, Laszlo D, et al. Addition of rituximab to chlorambucil produces superior event-free survival in the treatment of patients with extranodal marginal-zone B-cell lymphoma: 5-year analysis of the IELSG-19 randomized study. J Clin Oncol. 2013;31:565-572.[Abstract]
67. Ambrosetti A, Zanotti R, Pattaro C, et al. Most cases of primary salivary mucosa-associated lymphoid tissue lymphoma are associated either with Sjogren syndrome or hepatitis C virus infection. Br J Haematol. 2004;126:43-49. [Abstract]
68. Uno T, Isobe K, Shikama N, et al. Radiotherapy for extranodal, marginal zone, B-cell lymphoma of mucosa-associated lymphoid tissue originating in the ocular adnexa: a multiinstitutional, retrospective review of 50 patients. Cancer. 2003;98:865-871.[Abstract][Full Text]
69. Agulnik M, Tsang R, Baker MA, et al. Malignant lymphoma of mucosa-associated lymphoid tissue of the lacrimal gland: case report and review of literature. Am J Clin Oncol. 2001;24:67-70.[Abstract]
70. Ferreri AJ, Guidoboni M, Ponzoni M, et al. Evidence for an association between Chlamydia psittaci and ocular adnexal lymphomas. J Natl Cancer Inst. 2004;96:586-594.[Abstract][Full Text]
71. Fiche M, Caprons F, Berger F, et al. Primary pulmonary non-Hodgkin's lymphomas. Histopathology. 1995;26:529-537.[Abstract]
72. Ali R, Ozkalemkas F, Ozcelik T, et al. Successful treatment of BALT lymphoma with combined chemotherapy. Thorax. 2003;58:368-369.[Abstract][Full Text]
73. de Jong D, Boot H, Taal B. Histologic grading with clinical relevance in gastric mucosa-associated lymphoid tissue (MALT) lymphoma. Recent Results Cancer Res. 2000;156:27-32.[Abstract]
74. Jang YJ, Lim SM, Lee I, et al. A Phase II study of (131)I-rituximab for patients with relapsed or refractory marginal zone lymphoma. Clin Med Insights Oncol. 2023;17:11795549231218082.[Abstract][Full Text]
75. Lo AC, Holloway CL, Savage KJ, et al. Radioimmunotherapy for orbital marginal zone lymphoma: a retrospective review. Leuk Lymphoma. 2022 May;63(5):1242-5.
76. Bertoni F, Conconi A, Capella C, et al. Molecular follow-up in gastric mucosa-associated lymphoid tissue lymphomas: early analysis of the LY03 cooperative trial. Blood. 2002;99:2541-2544.[Abstract][Full Text]
77. Zullo A, Hassan C, Cristofari F,et al. Effects of Helicobacter pylori eradication on early stage gastric mucosa-associated lymphoid tissue lymphoma. Clin Gastroenterol Hepatol. 2010;8:105-110.[Abstract]
78. Zucca E, Cavalli F. Are antibiotics the treatment of choice for gastric lymphoma? Curr Hematol Rep. 2004;3:11-16.[Abstract]
79. Thieblemont C. Clinical presentation and management of marginal zone lymphomas. Hematology Am Soc Hematol Educ Program. 2005:307-313.[Abstract][Full Text]
