Opinion statement
Mucosa-associated lymphoid tissue (MALT) lymphomas occur in a variety of organs, including the orbit, conjunctiva, salivary glands, skin, thyroid gland, lungs, stomach, and intestine. These tumors are often localized and of indolent clinical behavior. Diagnosis is made by pathologic evaluation of a tissue biopsy. Careful staging is mandatory and tailored to the initial presentation. Staging includes a history and physical, chemistries, computed tomography scan, and bone marrow biopsy. This information is supplemented with an ear, nose, and throat consultation, esophagogastro-duodenoscopy, colonoscopy, endoscopic ultrasound of the stomach, and cytogenetic/immunohistochemical analysis of the tumors. Treatment is tailored to organ involvement and stage at presentation. Eradication of Helicobacter pylori using a triple anti-H. pylori regimen approved by the US Food and Drug Administration is standard therapy for all H. pyloripositive gastric MALT lymphomas. Endoscopic ultrasound- and computed tomographystaged gastric MALT stage IE tumors will achieve a complete response with this approach in approximately 60% to 90% of patients (the more superficial the tumor [T1/T2], the better the response). Patients with tumors that are T4 node-positive Musshoff stage IIE1 and IIE2 or tumors with adverse cytogenetics should receive radiotherapy or surgery with or without radiotherapy. Tumors with a significant high-grade component or large cell tumors with a minor low-grade MALT component should receive CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone)-based chemotherapy. Localized MALT lymphomas of the orbit, conjunctiva, salivary glands, and thyroid gland are treated successfully with radiotherapy. Surgery as first-line therapy for gastric MALT lymphomas was replaced by attempts at organ preservation. In the past, margin-free surgical excision or tumor debulking followed by radiation therapy and chemotherapy has been highly effective for gastric MALT lymphomas. Therefore, surgical excision of large cell or bulky tumors of the stomach, thyroid, lung, and salivary gland, followed by adjuvant radiotherapy or chemotherapy, may still be an important consideration in selected patients. Surgery still has a role for patients with relapsed or refractory lowgrade disease and life-threatening hemorrhage. Disseminated MALT lymphomas are incurable and are treated primarily with chemotherapy according to symptoms.
Similar content being viewed by others
References and Recommended Reading
Du MQ, Isaccson PG: Gastric MALT lymphoma: from aetiology to treatment. Lancet Oncol 2002, 3:97–104. An outstanding recent review of gastric MALT lymphoma etiology and treatment.
Wotherspoon AC, Dogan A, Du MQ: Mucosa-associated lymphoid tissue lymphoma. Curr Opin Hematol 2002, 9:50–55. An excellent review of MALT lymphoma pathogenesis, molecular pathology, and therapy.
Zucca E, Bertoni F, Roggero E, et al.: The gastric marginal zone B-cell lymphoma of MALT type. Blood 2000, 96:410–419. A very detailed review of pathogenesis, pathology, clinical features, and therapy of gastric MALT lymphomas.
Cavalli F, Isaacson PG, Gascoyne RD, et al.: MALT lymphomas. Hematology (Am Soc Hematol Educ Program) 2001, 241–258.
Raderer M, Vorbeck F, Formanek M, et al.: Importance of extensive staging in patients with mucosa-associated lymphoid tissue (MALT)-type lymphoma. Br J Cancer 2000, 83:454–457.
Schechter NR, Yahalom J: Low-grade MALT lymphoma of the stomach: a review of treatment options. Int J Radiat Oncol Biol Phys 2000, 46:1093–1103.
Ferreri AJ, Freschi M, Dell’Oro S, et al.: Prognostic significance of the histopathologic recognition of low- and high-grade components in stage I-II B-cell gastric lymphomas. Am J Surg Pathol 2001, 25:95–102.
de Jong D, Vyth-Dreese F, Dellemijn T, et al.: Histological and immunological parameters to predict treatment outcome of Helicobacter pylori eradication in low-grade gastric MALT lymphoma. J Pathol 2001, 193:318–24.
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.
Liu H, Ruskon-Fourmestraux A, Lavergne-Slove A, et al.: Resistance of t(11;18) positive gastric mucosa-associated lymphoid tissue lymphoma to Helicobacter pylori eradication therapy. Lancet 2001, 357:39–40. An important study demonstrating that most gastric MALT lymphomas with t(11;18) do not respond to H. pylori eradication therapy.
Wotherspoon AC: A Critical review of the effect of Helicobacter pylori eradication on gastric MALT lymphoma. Curr Gastroenterol Rep 2000, 2:494–498.
Bayerdorffer E, Neubauer A, Rudolph B, et al.: Regression of primary gastric lymphoma of mucosa-associated lymphoid tissue type after cure of Helicobacter pylori infection. MALT Lymphoma Study Group. Lancet 1995, 345:1591–1594.
Roggero E, Zucca E, Pinotti G, et al.: Eradication of Helicobacter pylori infection in primary low-grade gastric lymphoma of mucosa-associated lymphoid tissue. Ann Intern Med 1995, 122:767–769.
Suerbaum S, Michetti P: Helicobacter pylori infection. N Engl J Med 2002, 347:1175–1186. An outstanding review of H. pylori pathogenesis, clinical features, and therapy.
Neubauer A, Thiede C, Morgner A, et al.: Cure of Helicobacter pylori infection and duration of remission of low-grade gastric mucosa-associated lymphoid tissue lymphoma. J Natl Cancer Inst 1997, 89:1350–1355.
Steinbach G, Ford R, Glober G, et al.: Antibiotic treatment of gastric lymphoma of mucosa-associated lymphoid tissue: an uncontrolled trial. Ann Intern Med 1999, 131:88–95.
Ruskone-Fourmestraux A, Lavergne A, Aegerter PH, et al.: Predictive factors for regression of gastric MALT lymphoma after anti-Helicobacter pylori treatment. Gut 2001, 48:297–303. An important study regarding the value of endoscopic ultrasound in predicting poor response to H. pylori eradication therapy in patients who are node positive.
Sackmann M, Morgner A, Rudolph B, et al.: Regression of gastric MALT lymphoma after eradication of Helicobacter pylori is predicted by endosonographic staging. MALT Lymphoma Study Group. Gastroenterology 1997, 113:1087–1090. An important study regarding the value of endoscopic ultrasound in predicting poor response to H. pylori eradication therapy in patients with gastric MALT lymphomas with disease stage greater than E1-1 (involvement beyond mucosa and submucosa).
Nakamura S, Matsumoto T, Suekane H, et al.: Predictive value of endoscopic ultrasonography for regression of gastric low grade and high grade MALT lymphomas after eradication of Helicobacter pylori. Gut 2001, 48:454–460. An important study regarding the value of endoscopic ultrasound in predicting poor response to H. pylori eradication therapy in patients with gastric MALT lymphomas with disease extending into the deep submucosa.
Thiede C, Wundisch T, Alpen B, et al.: Long-term persistence of monoclonal B cells after cure of Helicobacter pylori infection and complete histologic remission in gastric mucosa-associated lymphoid tissue B-cell lymphoma. J Clin Oncol 2001, 19:1600–1609.
Fischbach W, Goebeler-Kolve M, Starostik P, et al.: Minimal residual low-grade gastric MALT-type lymphoma after eradication of Helicobacter pylori. Lancet 2002, 360:547–548.
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. Complete regression of gastric MALT lymphomas and PCR determinations of minimal residual disease do not correlate and minimal residual disease does not predict relapse. Caution is advised because follow-up time is short.
Zinzani PL, Magagnoli M, Galieni P, et al.: Nongastrointestinal low-grade mucosa-associated lymphoid tissue lymphoma: analysis of 75 patients. J Clin Oncol 1999, 17:1254.
Habermann TM, Ryu JH, Inwards DJ, et al.: Primary pulmonary lymphoma. Semin Oncol 1999, 26:307–315.
Ahmed S, Siddiqui AK, Rai KR: Low-grade B-cell bronchial associated lymphoid tissue (BALT) lymphoma. Cancer Invest 2002, 20:1059–1068.
Kurtin PJ, Myers JL, Adlakha H, et al.: Pathologic and clinical features of primary pulmonary extranodal marginal zone B-cell lymphoma of MALT type. Am J Surg Pathol 2001, 25:997–1008.
Blasi MA, Gherlinzoni F, Calvisi G, et al.: Local chemotherapy with interferon-alpha for conjunctival mucosa-associated lymphoid tissue lymphoma: a preliminary report. Ophthalmology 2001, 108:559–562. An important early observation that injection of interferon into the conjunctiva can cure conjunctival MALT lymphomas.
Thieblemont C, Berger F, Dumontet C, et al.: Mucosaassociated lymphoid tissue lymphoma is a disseminated disease in one third of 158 patients analyzed. Blood 2000, 95:802–806.
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.
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.
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 Biol Phys 2002, 52:1058–1066.
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, 50:1258–1264.
Le QT, Eulau SM, George TI, et al.: Primary radiotherapy for localized orbital MALT lymphoma. Int J Radiat Oncol Biol Phys 2002, 52:657–663.
Schechter NR, Portlock CS, Yahalom J: Treatment of mucosa-associated lymphoid tissue lymphoma of the stomach with radiation alone. J Clin Oncol 1998, 16:1916–1921.
Laing RW, Hoskin P, Hudson BV, et al.: The significance of MALT histology in thyroid lymphoma: a review of patients from the BNLI and Royal Marsden Hospital. Clin Oncol (R Coll Radiol) 1994, 6:300–304.
Nakamura S, Akazawa K, Yao T, et al.: A clinicopathologic study of 233 cases with special reference to evaluation with the MIB-1 index. Cancer 1995, 76:1313–1324.
Chen LT, Lin JT, Shyu RY, et al.: Prospective study of Helicobacter pylori eradication therapy in stage I(E) high-grade mucosa-associated lymphoid tissue lymphoma of the stomach. J Clin Oncol 2001, 19:4245–4251.
Morgner A, Miehlke S, Fischbach W, et al.: Complete remission of primary high-grade B-cell gastric lymphoma after cure of Helicobacter pylori infection. J Clin Oncol 2001, 19:2041–2048.
Ruskone-Fourmestraux A, Aegerter P, Delmer A, et al.: Primary digestive tract lymphoma: a prospective multicentric study of 91 patients. Groupe d’Etude des Lymphomes Digestifs. Gastroenterology 1993, 105:1662–1671.
Montalban C, Castrillo JM, Abraira V, et al.: Gastric Bcell mucosa-associated lymphoid tissue (MALT) lymphoma. Clinicopathological study and evaluation of the prognostic factors in 143 patients. Ann Oncol 1995, 6:355–362.
Popescu RA, Wotherspoon AC, Cunningham D, et al.: Surgery plus chemotherapy or chemotherapy alone for primary intermediate- and high-grade gastric non-Hodgkin’s lymphoma: the Royal Marsden Hospital experience. Eur J Cancer 1999, 35:928–934.
Ranaldi R, Goteri G, Baccarini MG, et al.: A clinicopathological study of 152 surgically treated primary gastric lymphomas with survival analysis of 109 high grade tumours. J Clin Pathol 2002, 55:346–351.
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Malek, S.N., Hatfield, A.J. & Flinn, I.W. MALT lymphomas. Curr. Treat. Options in Oncol. 4, 269–279 (2003). https://doi.org/10.1007/s11864-003-0002-2
Issue Date:
DOI: https://doi.org/10.1007/s11864-003-0002-2