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| __NOTOC__ | | __NOTOC__ |
| {{MALT lymphoma}} | | {{MALT lymphoma}} |
| {{CMG}}{{AE}}{{SR}} | | {{CMG}}{{AE}}{{SR}}, {{AY}} |
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| ==Overview== | | ==Overview== |
| MALT lymphoma is a form of [[non-Hodgkin lymphoma]] (NHL) involving the [[mucosa-associated lymphoid tissue]] (MALT), frequently of the [[stomach]], but virtually any mucosal site can be affected. It is a [[cancer]] originating from the [[B-cell]]s in the [[marginal zone]] of the MALT. The evolution of gastric MALT lymphoma is a multistage process starting with the infection of ''[[helicobacter|H. pylori]]'' resulting in the recruitment of B- and T-cells and other inflammatory cells to the gastric mucosa.<ref name="TroppanWenzl2015">{{cite journal|last1=Troppan|first1=Katharina|last2=Wenzl|first2=Kerstin|last3=Neumeister|first3=Peter|last4=Deutsch|first4=Alexander|title=Molecular Pathogenesis of MALT Lymphoma|journal=Gastroenterology Research and Practice|volume=2015|year=2015|pages=1–10|issn=1687-6121|doi=10.1155/2015/102656}}</ref> Genes involved in the pathogenesis of MALT lymphoma include ''[[FOXP1]]'' and [[BCL6]]. Chromosomal [[translocations]] are also involved in the pathogenesis of MALT lymphoma, which include t(1;14)(p22;q32), t(11;18)(q21;q21), t(14;18)(q32;q21), and t(3;14)(p14.1;q32). Gastric MALT lymphoma is frequently associated with chronic [[inflammation]] as a result of the presence of ''[[Helicobacter pylori]]'' (72-98%).<ref name=bjhjh>{{cite journal | author = Parsonnet J, Hansen S, Rodriguez L, Gelb A, Warnke R, Jellum E, Orentreich N, Vogelman J, Friedman G | title = Helicobacter pylori infection and gastric lymphoma. | journal = N Engl J Med | volume = 330 | issue = 18 | pages = 1267-71 | year = 1994 | id = PMID 8145781}}</ref> Chronic immune stimulation is also suspected in the pathogenesis of non-gastric MALT lymphoma, and hence often have a history of autoimmune disorders, such as [[Hashimoto's thyroiditis]], [[Sjögren's syndrome]], [[Celiac disease]], and [[relapsing polychondritis]].<ref name="KinkadeEsan2015">{{cite journal|last1=Kinkade|first1=Zoe|last2=Esan|first2=Olukemi A.|last3=Rosado|first3=Flavia G.|last4=Craig|first4=Michael|last5=Vos|first5=Jeffrey A.|title=Ileal mucosa-associated lymphoid tissue lymphoma presenting with small bowel obstruction: a case report|journal=Diagnostic Pathology|volume=10|issue=1|year=2015|issn=1746-1596|doi=10.1186/s13000-015-0353-6}}</ref> MALT lymphoma starts in the tissues or organs outside of the lymph nodes (''extranodal''). MALT lymphoma develops in mucosa-associated lymphoid tissue, in the mucosa, or tissue that lines body organs or body cavities including [[gastrointestinal tract]], [[lungs]], [[orbit]], [[skin]], [[salivary glands]], [[thyroid gland]], and [[breasts]].<ref name="KinkadeEsan2015">{{cite journal|last1=Kinkade|first1=Zoe|last2=Esan|first2=Olukemi A.|last3=Rosado|first3=Flavia G.|last4=Craig|first4=Michael|last5=Vos|first5=Jeffrey A.|title=Ileal mucosa-associated lymphoid tissue lymphoma presenting with small bowel obstruction: a case report|journal=Diagnostic Pathology|volume=10|issue=1|year=2015|issn=1746-1596|doi=10.1186/s13000-015-0353-6}}</ref><ref name=extranodalmarginalzoneofmucosaassociatedlymphoidtissueMALTlymphoma1>Extranodal marginal zone of mucosa-associated lymphoid tissue (MALT lymphoma). Canadian Cancer Society 2016. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/malt-lymphoma/?region=on. Accessed on January 28, 2016</ref> On microscopic histopathological analysis, MALT lymphoma is characterized by the presence of dense diffuse lymphoid infiltrate of marginal‐zone cells in lamina propria with prominent lymphoepithelial lesions and consisting of small atypical cells with monocytoid features.<ref>{{cite journal|last1=Taal|first1=B G|last2=Boot|first2=H|last3=van Heerde|first3=P|last4=de Jong|first4=D|last5=Hart|first5=A A|last6=Burgers|first6=J M|title=Primary non-Hodgkin lymphoma of the stomach: endoscopic pattern and prognosis in low versus high grade malignancy in relation to the MALT concept.|journal=Gut|date=1 October 1996|volume=39|issue=4|pages=556–561|doi=10.1136/gut.39.4.556}}</ref><ref name="pmid16950858">{{cite journal| author=Bacon CM, Du MQ, Dogan A| title=Mucosa-associated lymphoid tissue (MALT) lymphoma: a practical guide for pathologists. | journal=J Clin Pathol | year= 2007 | volume= 60 | issue= 4 | pages= 361-72 | pmid=16950858 | doi=10.1136/jcp.2005.031146 | pmc=PMC2001121 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16950858 }} </ref> A characteristic feature of MALT lymphoma is the presence of neoplastic cells within epithelial structures with associated destruction of the glandular architecture to form lymphoepithelial lesions.<ref>{{cite book|last1=Janusz|first1=edited by Jankowski,|title=Handbook of Gastrointestinal Cancer|date=2012|publisher=John Wiley and Sons Ltd|location=Chicester|isbn=978-0-470-65624-2|pages=243–244|edition=2}}</ref> The neoplastic cells of MALT lymphoma may be positive for B-cell associated antigens ([[CD19]], [[CD20]], [[CD22]], [[CD79a]]) that co-express [[BCL-2]], and are negative for [[CD5]], [[CD10]], [[CD43]], and [[cyclin D1]].<ref name="KinkadeEsan2015">{{cite journal|last1=Kinkade|first1=Zoe|last2=Esan|first2=Olukemi A.|last3=Rosado|first3=Flavia G.|last4=Craig|first4=Michael|last5=Vos|first5=Jeffrey A.|title=Ileal mucosa-associated lymphoid tissue lymphoma presenting with small bowel obstruction: a case report|journal=Diagnostic Pathology|volume=10|issue=1|year=2015|issn=1746-1596|doi=10.1186/s13000-015-0353-6}}</ref> MALT lymphoma must be differentiated from [[gastric carcinoma]], [[gastritis]], [[diffuse large B cell lymphoma]], [[follicular lymphoma]], [[small cell lymphocytic lymphoma]], and [[mantle cell lymphoma]].<ref name=ddxgastriclummjj1>Differential diagnosis of gastric lymphoma. Dr Henry Knipe and A.Prof Frank Gaillard et al. Radiopaedia 2016. http://radiopaedia.org/articles/gastric-lymphoma. Accessed on January 29, 2016</ref><ref>{{cite journal |last=Smith |first=LB|last=Owens|first=SR |year=2012 |title=Gastrointestinal Lymphomas Entities and Mimics |publisher=Arch Pathol Lab Med. 2012 |url=http://www.archivesofpathology.org/doi/pdf/10.5858/arpa.2012-0211-CR |accessdate=02/01/2016 }}</ref><ref name="pmid16950858">{{cite journal| author=Bacon CM, Du MQ, Dogan A| title=Mucosa-associated lymphoid tissue (MALT) lymphoma: a practical guide for pathologists. | journal=J Clin Pathol | year= 2007 | volume= 60 | issue= 4 | pages= 361-72 | pmid=16950858 | doi=10.1136/jcp.2005.031146 | pmc=PMC2001121 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16950858 }} </ref> MALT lymphoma is the third most common type and accounts for approximately 8% of all cases of [[non-Hodgkin lymphoma]].<ref name=extranodalmarginalzoneofmucosaassociatedlymphoidtissueMALTlymphoma1>Extranodal marginal zone of mucosa-associated lymphoid tissue (MALT lymphoma). Canadian Cancer Society 2016. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/malt-lymphoma/?region=on. Accessed on January 28, 2016</ref> MALT lymphoma is a disease that tends to affect the elderly population. Most MALT lymphomas occur in people in their 60's.<ref name=extranodalmarginalzoneofmucosaassociatedlymphoidtissueMALTlymphoma1>Extranodal marginal zone of mucosa-associated lymphoid tissue (MALT lymphoma). Canadian Cancer Society 2016. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/malt-lymphoma/?region=on. Accessed on January 28, 2016</ref> Males are more commonly affected with gastric MALT lymphoma than females. The male to female ratio is approximately 2.43 to 1.<ref name="pmid21419026">{{cite journal| author=Pervez S, Ali N, Aaqil H, Mumtaz K, Siddiq Ullah S, Akhtar N| title=Gastric MALT lymphoma: a rarity. | journal=J Coll Physicians Surg Pak | year= 2011 | volume= 21 | issue= 3 | pages= 171-2 | pmid=21419026 | doi=03.2011/JCPSP.171172 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21419026 }} </ref> There is no racial predilection to MALT lymphoma.<ref name=racemaltlymphoma1>What is the Incidence of MALT. Lymphomation.org 2016. http://www.lymphomation.org/type-malt.htm. Accessed on February 1, 2016</ref> MALT lymphoma is usually slow growing (indolent), but some can be high grade.<ref name=extranodalmarginalzoneofmucosaassociatedlymphoidtissueMALTlymphoma1>Extranodal marginal zone of mucosa-associated lymphoid tissue (MALT lymphoma). Canadian Cancer Society 2016. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/malt-lymphoma/?region=on. Accessed on January 28, 2016</ref> If left untreated, MALT lymphoma may progress to develop [[fever]], [[anemia]], [[weight loss]], and large cell lymphoma.<ref name=extranodalmarginalzoneofmucosaassociatedlymphoidtissueMALTlymphoma1>Extranodal marginal zone of mucosa-associated lymphoid tissue (MALT lymphoma). Canadian Cancer Society 2016. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/malt-lymphoma/?region=on. Accessed on January 28, 2016</ref> Complications of MALT lymphoma include [[anemia]], [[cachexia]], [[obstruction]], and [[Perforation|perforation of small intestine]].<ref name=extranodalmarginalzoneofmucosaassociatedlymphoidtissueMALTlymphoma1>Extranodal marginal zone of mucosa-associated lymphoid tissue (MALT lymphoma). Canadian Cancer Society 2016. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/malt-lymphoma/?region=on. Accessed on January 28, 2016</ref> The prognosis is good, and the 10-year survival rate for gastric MALT lymphoma is approximately 90% with a disease-free survival of approximately 70%.<ref name="TroppanWenzl2015">{{cite journal|last1=Troppan|first1=Katharina|last2=Wenzl|first2=Kerstin|last3=Neumeister|first3=Peter|last4=Deutsch|first4=Alexander|title=Molecular Pathogenesis of MALT Lymphoma|journal=Gastroenterology Research and Practice|volume=2015|year=2015|pages=1–10|issn=1687-6121|doi=10.1155/2015/102656}}</ref> However, in rare instances, MALT lymphoma can progress and transform into aggressive high-grade tumors, such as extranodal diffuse large B cell lymphoma (eDLBCL), whereby the 10-year survival rate drops to approximately 42%. The staging of MALT lymphoma is based on two staging systems: Ann Arbor staging system and the TNM staging system.<ref name=stagingmaltlymphoma1>Staging MALT lymphoma of the stomach. Canadian Cancer Society 2016. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/malt-lymphoma/?region=on. Accessed on January 25, 2016</ref> Symptoms of MALT lymphoma include [[pigastric pain]], [[fatigue]], [[heartburn]], [[fever]], [[cough]] with [[hemoptysis|blood in sputum]], [[dyspnea|shortness of breath]], and/or [[Orbital mass causes|lump in the superior lateral quadrant of the orbit]], [[breast]], [[neck]], or [[salivary gland]].<ref name=sympromaltlymphoma2>Symptoms of MALT lymphoma. Cancer research UK 2016. http://www.cancerresearchuk.org/about-cancer/type/non-hodgkins-lymphoma/about/types/mucosaassociated-lymphoid-tissue-lymphoma. Accessed on January 28, 2016</ref><ref name=sympromaltlymphoma1>Signs and symptoms of gastric lymphoma. Wikipedia 2016. https://en.wikipedia.org/wiki/Gastric_lymphoma. Accessed on January 28, 2016</ref><ref name=nongastricorbitlymphomasym1>Clinical presentation of orbital lymphoma. Dr Craig Hacking and A.Prof Frank Gaillard et al. Radiopaedia 2016. http://radiopaedia.org/articles/orbital-lymphoma. Accessed on January 28, 2016</ref><ref name=sympmalylymmnoingastry>Non-gastric lymphomas – causes, symptoms and treatments. Lymphoma association 2016. https://www.lymphomas.org.uk/sites/default/files/pdfs/Non-Gastric-malt-lymphoma.pdf. Accessed on January 28, 2016</ref> [[Complete blood count]] is performed to check the blood counts.<ref name=sympmalylymmnoingastry>Non-gastric lymphomas – causes, symptoms and treatments. Lymphoma association 2016. https://www.lymphomas.org.uk/sites/default/files/pdfs/Non-Gastric-malt-lymphoma.pdf. Accessed on January 28, 2016</ref> [[Liver function tests]] and [[renal function tests]] are performed to assess the liver and kidneys, respectively.<ref name=sympmalylymmnoingastry>Non-gastric lymphomas – causes, symptoms and treatments. Lymphoma association 2016. https://www.lymphomas.org.uk/sites/default/files/pdfs/Non-Gastric-malt-lymphoma.pdf. Accessed on January 28, 2016</ref> The gastric lesion of MALT lymphoma on [[esophagogastroduodenoscopy|upper GI endoscopy]] may look inflammed or ulcerated. Sometimes, a nodular mass, similar to a reactive lymph node, may be visualized.<ref name=sympmalylymmnoingastry>Non-gastric lymphomas – causes, symptoms and treatments. Lymphoma association 2016. https://www.lymphomas.org.uk/sites/default/files/pdfs/Non-Gastric-malt-lymphoma.pdf. Accessed on January 28, 2016</ref><ref name="pmid22943012">{{cite journal| author=Bautista-Quach MA, Ake CD, Chen M, Wang J| title=Gastrointestinal lymphomas: Morphology, immunophenotype and molecular features. | journal=J Gastrointest Oncol | year= 2012 | volume= 3 | issue= 3 | pages= 209-25 | pmid=22943012 | doi=10.3978/j.issn.2078-6891.2012.024 | pmc=PMC3418529 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22943012 }} </ref> Chest, abdominal, and/or pelvic CT and MRI scan are generally performed to evaluate the extent and spread of MALT lymphoma.<ref name="pmid23879202">{{cite journal| author=Nasser QJ, Pfeiffer ML, Romaguera J, Fowler N, Debnam JM, Samaniego F et al.| title=Clinical value of magnetic resonance imaging and other baseline testing for conjunctival mucosa-associated lymphoid tissue lymphoma. | journal=Leuk Lymphoma | year= 2014 | volume= 55 | issue= 5 | pages= 1013-7 | pmid=23879202 | doi=10.3109/10428194.2013.826353 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23879202 }} </ref> If the disease is limited to the stomach (which is assessed with [[computed tomography]]), then 70-80% of patients will have a complete regression on treatment with [[antibiotic]] eradication of ''H. pylori''.<ref name=vujhg>{{cite journal | author = Bayerdörffer E, Neubauer A, Rudolph B, Thiede C, Lehn N, Eidt S, Stolte M | title = Regression of primary gastric lymphoma of mucosa-associated lymphoid tissue type after cure of Helicobacter pylori infection. MALT Lymphoma Study Group. | journal = Lancet | volume = 345 | issue = 8965 | pages = 1591-4 | year = 1995 | id = PMID 7783535}}</ref> Endoscopic ultrasound may be performed to detect the extent of spread of MALT lymphoma through the stomach wall. The endoscopic ultrasound scan may demonstrate the deeper tissues that lie to the stomach lining and also involvement of any adjacent lymph nodes.<ref name=sympmalylymmnoingastry>Non-gastric lymphomas – causes, symptoms and treatments. Lymphoma association 2016. https://www.lymphomas.org.uk/sites/default/files/pdfs/Non-Gastric-malt-lymphoma.pdf. Accessed on January 28, 2016</ref> Other imaging studies for MALT lymphoma include barium studies and [[PET scan]]. Barium contrast studies of the esophagus, stomach, small intestine, or the colon may be performed to demonstrate any filling defect in the barium flow which denotes to the presence of any infiltrative mass due to MALT lymphoma.<ref name="pmid16127783">{{cite journal| author=Chung JJ, Kim MJ, Kie JH, Kim KW| title=Mucosa-associated lymphoid tissue lymphoma of the esophagus coexistent with bronchus-associated lymphoid tissue lymphoma of the lung. | journal=Yonsei Med J | year= 2005 | volume= 46 | issue= 4 | pages= 562-6 | pmid=16127783 | doi= | pmc=PMC2815843 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16127783 }} </ref> [[FDG-PET|FDG-PET scan]] may be helpful in diagnosis, staging, and follow-up of MALT lymphoma.<ref name="Beal2005">{{cite journal|last1=Beal|first1=K. P.|title=FDG-PET scanning for detection and staging of extranodal marginal zone lymphomas of the MALT type: a report of 42 cases|journal=Annals of Oncology|volume=16|issue=3|year=2005|pages=473–480|issn=0923-7534|doi=10.1093/annonc/mdi093}}</ref><ref name="pmid17662066">{{cite journal| author=Perry C, Herishanu Y, Metzer U, Bairey O, Ruchlemer R, Trejo L et al.| title=Diagnostic accuracy of PET/CT in patients with extranodal marginal zone MALT lymphoma. | journal=Eur J Haematol | year= 2007 | volume= 79 | issue= 3 | pages= 205-9 | pmid=17662066 | doi=10.1111/j.1600-0609.2007.00895.x | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17662066 }} </ref> The optimal therapy for MALT lymphoma depends on the stage at diagnosis. The treatment options for early stage (localized) gastric MALT lymphoma include [[antibiotic therapy]], [[radiotherapy]], [[chemotherapy]], [[surgery]], and [[monoclonal antibodies]].<ref name=treatmentofMALTlymphomaofthestomachrx1>Treatment of MALT lymphoma of the stomach. Canadian Cancer Society 2016. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/malt-lymphoma/?region=on. Accessed on January 28, 2016</ref> The treatment options for advanced stage gastric MALT lymphoma include observation, [[radiotherapy]], [[chemotherapy]], and [[monoclonal antibodies]].<ref name=treatmentofMALTlymphomaofthestomachrx1>Treatment of MALT lymphoma of the stomach. Canadian Cancer Society 2016. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/malt-lymphoma/?region=on. Accessed on January 28, 2016</ref> Secondary prevention strategies following MALT lymphoma include [[urease breath test]], performed 4-6 weeks after completion of the course of antibiotic therapy, and [[endoscopy]] with concurrent [[biopsy]], performed 3-6 months after the treatment is finished.<ref name=sympmalylymmnoingastry>Non-gastric lymphomas – causes, symptoms and treatments. Lymphoma association 2016. https://www.lymphomas.org.uk/sites/default/files/pdfs/Non-Gastric-malt-lymphoma.pdf. Accessed on January 28, 2016</ref> | | MALT lymphoma is a common form of [[non-Hodgkin lymphoma]] (NHL) involving the [[mucosa-associated lymphoid tissue]] (MALT), frequently of the [[stomach]], but virtually any mucosal site can be affected. It is a [[cancer]] originating from the [[B-cell]]s in the [[marginal zone]] of the MALT. The evolution of gastric MALT lymphoma is a multistage process starting with the infection of ''[[helicobacter|H. pylori]]'' resulting in the recruitment of B- and T-cells and other inflammatory cells to the gastric mucosa. Genes involved in the pathogenesis of MALT lymphoma include ''[[FOXP1]]'' and [[BCL6]]. A characteristic feature of MALT lymphoma is the presence of neoplastic cells within epithelial structures with associated destruction of the glandular architecture to form lymphoepithelial lesions. Most MALT lymphomas occur among individuals of 60 years of age. Males are more commonly affected with gastric MALT lymphoma than females. MALT lymphoma is usually slow growing (indolent), but some can be high grade. If left untreated, MALT lymphoma may progress to develop [[fever]], [[anemia]], [[weight loss]], and large cell lymphoma. Complications of MALT lymphoma include [[anemia]], [[cachexia]], [[obstruction]], and [[Perforation|perforation of small intestine]]. The prognosis is good, and the 10-year survival rate for gastric MALT lymphoma is approximately 90% with a disease-free survival of approximately 70%. However, in rare instances, MALT lymphoma can progress and transform into aggressive high-grade tumors, such as extranodal diffuse large B cell lymphoma (eDLBCL), whereby the 10-year survival rate drops to approximately 42%. The staging of MALT lymphoma is based on two staging systems: Ann Arbor staging system and the TNM staging system. If the disease is limited to the stomach (which is assessed with [[computed tomography]]), then 70-80% of patients will have a complete regression on treatment with [[antibiotic]] eradication of ''H. pylori''. The optimal therapy for MALT lymphoma depends on the stage at diagnosis. The treatment options for early stage (localized) gastric MALT lymphoma include [[antibiotic therapy]], [[radiotherapy]], [[chemotherapy]], [[surgery]], and [[monoclonal antibodies]]. The treatment options for advanced stage gastric MALT lymphoma include observation, [[radiotherapy]], [[chemotherapy]], and [[monoclonal antibodies]]. Secondary prevention strategies following MALT lymphoma include [[urease breath test]], performed 4-6 weeks after completion of the course of antibiotic therapy, and [[endoscopy]] with concurrent [[biopsy]], performed 3-6 months after the treatment is finished. |
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| ==Historical Perspective== | | ==Historical Perspective== |
| MALT lymphoma was first described by Isaacson and Wright, in 1983.<ref name="TroppanWenzl2015">{{cite journal|last1=Troppan|first1=Katharina|last2=Wenzl|first2=Kerstin|last3=Neumeister|first3=Peter|last4=Deutsch|first4=Alexander|title=Molecular Pathogenesis of MALT Lymphoma|journal=Gastroenterology Research and Practice|volume=2015|year=2015|pages=1–10|issn=1687-6121|doi=10.1155/2015/102656}}</ref> | | MALT lymphoma was first described by Isaacson and Wright in 1983. |
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| ==Classification== | | ==Classification== |
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| ==Pathophysiology== | | ==Pathophysiology== |
| MALT lymphoma is a form of [[lymphoma]] involving the [[mucosa-associated lymphoid tissue]] (MALT), frequently of the [[stomach]], but virtually any mucosal site can be afflicted. It is a [[cancer]] originating from [[B cell]]s in the [[marginal zone]] of the MALT. The evolution of gastric MALT lymphoma is a multistage process starting with the infection of ''[[helicobacter|H. pylori]]'' resulting in the recruitment of B- and T-cells and other inflammatory cells to the gastric mucosa.<ref name="TroppanWenzl2015">{{cite journal|last1=Troppan|first1=Katharina|last2=Wenzl|first2=Kerstin|last3=Neumeister|first3=Peter|last4=Deutsch|first4=Alexander|title=Molecular Pathogenesis of MALT Lymphoma|journal=Gastroenterology Research and Practice|volume=2015|year=2015|pages=1–10|issn=1687-6121|doi=10.1155/2015/102656}}</ref> Genes involved in the pathogenesis of MALT lymphoma include ''[[FOXP1]]'' and [[BCL6]]. Chromosomal [[translocations]] are also involved in the pathogenesis of MALT lymphoma, which include t(1;14)(p22;q32), t(11;18)(q21;q21), t(14;18)(q32;q21), and t(3;14)(p14.1;q32). Gastric MALT lymphoma is frequently associated with chronic [[inflammation]] as a result of the presence of ''[[Helicobacter pylori]]'' (72-98%).<ref name=bjhjh>{{cite journal | author = Parsonnet J, Hansen S, Rodriguez L, Gelb A, Warnke R, Jellum E, Orentreich N, Vogelman J, Friedman G | title = Helicobacter pylori infection and gastric lymphoma. | journal = N Engl J Med | volume = 330 | issue = 18 | pages = 1267-71 | year = 1994 | id = PMID 8145781}}</ref> Chronic immune stimulation is also suspected in the pathogenesis of non-gastric MALT lymphoma, and hence often have a history of autoimmune disorders, such as [[Hashimoto's thyroiditis]], [[Sjögren's syndrome]], [[Celiac disease]], and [[relapsing polychondritis]].<ref name="KinkadeEsan2015">{{cite journal|last1=Kinkade|first1=Zoe|last2=Esan|first2=Olukemi A.|last3=Rosado|first3=Flavia G.|last4=Craig|first4=Michael|last5=Vos|first5=Jeffrey A.|title=Ileal mucosa-associated lymphoid tissue lymphoma presenting with small bowel obstruction: a case report|journal=Diagnostic Pathology|volume=10|issue=1|year=2015|issn=1746-1596|doi=10.1186/s13000-015-0353-6}}</ref> MALT lymphoma starts in the tissues or organs outside of the lymph nodes (''extranodal''). MALT lymphoma develops in mucosa-associated lymphoid tissue, in the mucosa, or tissue that lines body organs or body cavities including [[gastrointestinal tract]], [[lungs]], [[orbit]], [[skin]], [[salivary glands]], [[thyroid gland]], and [[breasts]].<ref name="KinkadeEsan2015">{{cite journal|last1=Kinkade|first1=Zoe|last2=Esan|first2=Olukemi A.|last3=Rosado|first3=Flavia G.|last4=Craig|first4=Michael|last5=Vos|first5=Jeffrey A.|title=Ileal mucosa-associated lymphoid tissue lymphoma presenting with small bowel obstruction: a case report|journal=Diagnostic Pathology|volume=10|issue=1|year=2015|issn=1746-1596|doi=10.1186/s13000-015-0353-6}}</ref><ref name=extranodalmarginalzoneofmucosaassociatedlymphoidtissueMALTlymphoma1>Extranodal marginal zone of mucosa-associated lymphoid tissue (MALT lymphoma). Canadian Cancer Society 2016. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/malt-lymphoma/?region=on. Accessed on January 28, 2016</ref> On microscopic histopathological analysis, MALT lymphoma is characterized by the presence of dense diffuse lymphoid infiltrate of marginal‐zone cells in lamina propria with prominent lymphoepithelial lesions and consisting of small atypical cells with monocytoid features.<ref>{{cite journal|last1=Taal|first1=B G|last2=Boot|first2=H|last3=van Heerde|first3=P|last4=de Jong|first4=D|last5=Hart|first5=A A|last6=Burgers|first6=J M|title=Primary non-Hodgkin lymphoma of the stomach: endoscopic pattern and prognosis in low versus high grade malignancy in relation to the MALT concept.|journal=Gut|date=1 October 1996|volume=39|issue=4|pages=556–561|doi=10.1136/gut.39.4.556}}</ref><ref name="pmid16950858">{{cite journal| author=Bacon CM, Du MQ, Dogan A| title=Mucosa-associated lymphoid tissue (MALT) lymphoma: a practical guide for pathologists. | journal=J Clin Pathol | year= 2007 | volume= 60 | issue= 4 | pages= 361-72 | pmid=16950858 | doi=10.1136/jcp.2005.031146 | pmc=PMC2001121 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16950858 }} </ref> A characteristic feature of MALT lymphoma is the presence of neoplastic cells within epithelial structures with associated destruction of the glandular architecture to form lymphoepithelial lesions.<ref>{{cite book|last1=Janusz|first1=edited by Jankowski,|title=Handbook of Gastrointestinal Cancer|date=2012|publisher=John Wiley and Sons Ltd|location=Chicester|isbn=978-0-470-65624-2|pages=243–244|edition=2}}</ref> The neoplastic cells of MALT lymphoma may be positive for B-cell associated antigens ([[CD19]], [[CD20]], [[CD22]], [[CD79a]]) that co-express [[BCL-2]], and are negative for [[CD5]], [[CD10]], [[CD43]], and [[cyclin D1]].<ref name="KinkadeEsan2015">{{cite journal|last1=Kinkade|first1=Zoe|last2=Esan|first2=Olukemi A.|last3=Rosado|first3=Flavia G.|last4=Craig|first4=Michael|last5=Vos|first5=Jeffrey A.|title=Ileal mucosa-associated lymphoid tissue lymphoma presenting with small bowel obstruction: a case report|journal=Diagnostic Pathology|volume=10|issue=1|year=2015|issn=1746-1596|doi=10.1186/s13000-015-0353-6}}</ref> | | MALT lymphoma is a form of [[lymphoma]] involving the [[mucosa-associated lymphoid tissue]] (MALT), frequently of the [[stomach]], but virtually any mucosal site can be afflicted. It is a [[cancer]] originating from [[B cell]]s in the [[marginal zone]] of the MALT. The evolution of gastric MALT lymphoma is a multistage process starting with the infection of ''[[helicobacter|H. pylori]]'' resulting in the recruitment of B- and T-cells and other inflammatory cells to the gastric mucosa. Genes involved in the pathogenesis of MALT lymphoma include ''[[FOXP1]]'' and [[BCL6]]. Chromosomal [[translocations]] are also involved in the pathogenesis of MALT lymphoma, which include t(1;14)(p22;q32), t(11;18)(q21;q21), t(14;18)(q32;q21), and t(3;14)(p14.1;q32). Gastric MALT lymphoma is frequently associated with chronic [[inflammation]] as a result of the presence of ''[[Helicobacter pylori]]'' (72-98%). On microscopic histopathological analysis, MALT lymphoma is characterized by the presence of dense diffuse lymphoid infiltrate of marginal‐zone cells in lamina propria with prominent lymphoepithelial lesions and consisting of small atypical cells with monocytoid features. A characteristic feature of MALT lymphoma is the presence of neoplastic cells within epithelial structures with associated destruction of the glandular architecture to form lymphoepithelial lesions. The neoplastic cells of MALT lymphoma may be positive for B-cell associated antigens ([[CD19]], [[CD20]], [[CD22]], [[CD79a]]) that co-express [[BCL-2]], and are negative for [[CD5]], [[CD10]], [[CD43]], and [[cyclin D1]]. |
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| ==Causes== | | ==Causes== |
| Common causes of gastric MALT lymphoma include [[helicobacter|''H.pyroli'' infection]], [[Immunosuppressive drug|long-term immunosuppressant drug therapy]], and [[HIV infection]].<ref name=riskfactorsmaltlymphoma1>Risks of Extranodal marginal zone of mucosa-associated lymphoid tissue (MALT lymphoma). Canadian Cancer Society 2016. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/malt-lymphoma/?region=on. Accessed on January 25, 2016</ref> Common causes of nongastric MALT lymphoma include [[autoimmune disorders]], such as [[Hashimoto's thyroiditis]] and [[Sjogren’s syndrome]].<ref name=riskfactorsmaltlymphoma1>Risks of Extranodal marginal zone of mucosa-associated lymphoid tissue (MALT lymphoma). Canadian Cancer Society 2016. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/malt-lymphoma/?region=on. Accessed on January 25, 2016</ref> | | Common causes of gastric MALT lymphoma include [[helicobacter|''H.pyroli'' infection]], [[Immunosuppressive drug|long-term immunosuppressant drug therapy]], and [[HIV infection]]. Common causes of non gastric MALT lymphoma include [[autoimmune disorders]], such as [[Hashimoto's thyroiditis]] and [[Sjogren’s syndrome]]. |
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| ==Differentiating MALT Lymphoma from other Diseases== | | ==Differentiating MALT Lymphoma from other Diseases== |
| MALT lymphoma must be differentiated from [[gastric carcinoma]], [[gastritis]], [[diffuse large B cell lymphoma]], [[follicular lymphoma]], [[small cell lymphocytic lymphoma]], and [[mantle cell lymphoma]].<ref name=ddxgastriclummjj1>Differential diagnosis of gastric lymphoma. Dr Henry Knipe and A.Prof Frank Gaillard et al. Radiopaedia 2016. http://radiopaedia.org/articles/gastric-lymphoma. Accessed on January 29, 2016</ref><ref>{{cite journal |last=Smith |first=LB|last=Owens|first=SR |year=2012 |title=Gastrointestinal Lymphomas Entities and Mimics |publisher=Arch Pathol Lab Med. 2012 |url=http://www.archivesofpathology.org/doi/pdf/10.5858/arpa.2012-0211-CR |accessdate=02/01/2016 }}</ref><ref name="pmid16950858">{{cite journal| author=Bacon CM, Du MQ, Dogan A| title=Mucosa-associated lymphoid tissue (MALT) lymphoma: a practical guide for pathologists. | journal=J Clin Pathol | year= 2007 | volume= 60 | issue= 4 | pages= 361-72 | pmid=16950858 | doi=10.1136/jcp.2005.031146 | pmc=PMC2001121 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16950858 }} </ref> | | MALT lymphoma must be differentiated from [[gastric carcinoma]], [[gastritis]], [[diffuse large B cell lymphoma]], [[follicular lymphoma]], [[small cell lymphocytic lymphoma]], and [[mantle cell lymphoma]]. |
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| ==Epidemiology and Demographics== | | ==Epidemiology and Demographics== |
| MALT lymphoma is the third most common type and accounts for approximately 8% of all cases of [[non-Hodgkin lymphoma]].<ref name=extranodalmarginalzoneofmucosaassociatedlymphoidtissueMALTlymphoma1>Extranodal marginal zone of mucosa-associated lymphoid tissue (MALT lymphoma). Canadian Cancer Society 2016. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/malt-lymphoma/?region=on. Accessed on January 28, 2016</ref> MALT lymphoma is a disease that tends to affect the elderly population. Most MALT lymphomas occur in people in their 60's.<ref name=extranodalmarginalzoneofmucosaassociatedlymphoidtissueMALTlymphoma1>Extranodal marginal zone of mucosa-associated lymphoid tissue (MALT lymphoma). Canadian Cancer Society 2016. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/malt-lymphoma/?region=on. Accessed on January 28, 2016</ref> Males are more commonly affected with gastric MALT lymphoma than females. The male to female ratio is approximately 2.43 to 1.<ref name="pmid21419026">{{cite journal| author=Pervez S, Ali N, Aaqil H, Mumtaz K, Siddiq Ullah S, Akhtar N| title=Gastric MALT lymphoma: a rarity. | journal=J Coll Physicians Surg Pak | year= 2011 | volume= 21 | issue= 3 | pages= 171-2 | pmid=21419026 | doi=03.2011/JCPSP.171172 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21419026 }} </ref> There is no racial predilection to MALT lymphoma.<ref name=racemaltlymphoma1>What is the Incidence of MALT. Lymphomation.org 2016. http://www.lymphomation.org/type-malt.htm. Accessed on February 1, 2016</ref> | | MALT lymphoma is the third most common type and accounts for approximately 8% of all cases of [[non-Hodgkin lymphoma]]. MALT lymphoma is a disease that tends to affect the elderly population. Most MALT lymphomas occur in people in their 60's. Males are more commonly affected with gastric MALT lymphoma than females. The male to female ratio is approximately 2.43 to 1. There is no racial predilection to MALT lymphoma. |
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| ==Risk Factors== | | ==Risk Factors== |
| The most potent risk factor in the development of gastric MALT lymphoma is [[helicobacter|''H.pyroli'' infection]]. About 5–10% of people with gastric MALT lymphoma do not have an ''H. pylori'' infection (''H. pylori'' negative).<ref name=riskfactorsmaltlymphoma1>Risks of Extranodal marginal zone of mucosa-associated lymphoid tissue (MALT lymphoma). Canadian Cancer Society 2016. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/malt-lymphoma/?region=on. Accessed on January 25, 2016</ref> Other risk factors for gastric MALT lymphoma include [[Immunosuppressive drug|long-term immunosuppressant drug therapy]] and [[HIV infection]].<ref name=riskfactorsmaltlymphoma2>Risk factors of gastric lymphoma. Wikipedia 2016. https://en.wikipedia.org/wiki/Gastric_lymphoma. Accessed on January 25, 2016</ref> Common risk factors in the development of MALT lymphoma, in areas of the body other than the stomach, are [[autoimmune disorders]] such as [[Hashimoto's thyroiditis]], [[Sjogren’s syndrome]], and [[Celiac disease]].<ref name=riskfactorsmaltlymphoma1>Risks of Extranodal marginal zone of mucosa-associated lymphoid tissue (MALT lymphoma). Canadian Cancer Society 2016. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/malt-lymphoma/?region=on. Accessed on January 25, 2016</ref> | | The most potent risk factor in the development of gastric MALT lymphoma is [[H.pylori infection diagnostic tests|''H.pylori'' infection]]. Approximately 5–10% of people with gastric MALT lymphoma, however, do not have an ''H. pylori'' infection (''H. pylori'' negative). Other risk factors for gastric MALT lymphoma include [[Immunosuppressive drug|long-term immunosuppressant drug therapy]] and [[HIV infection]]. Common risk factors in the development of MALT lymphoma, in areas of the body other than the stomach, are [[autoimmune disorders]] such as [[Hashimoto's thyroiditis]], [[Sjogren’s syndrome]], and [[Celiac disease]]. |
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| ==Screening== | | ==Screening== |
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| ==Natural History, Complications and Prognosis== | | ==Natural History, Complications and Prognosis== |
| MALT lymphoma is usually slow growing (indolent), but some can be high grade.<ref name=extranodalmarginalzoneofmucosaassociatedlymphoidtissueMALTlymphoma1>Extranodal marginal zone of mucosa-associated lymphoid tissue (MALT lymphoma). Canadian Cancer Society 2016. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/malt-lymphoma/?region=on. Accessed on January 28, 2016</ref> If left untreated, MALT lymphoma may progress to develop [[fever]], [[anemia]], [[weight loss]], and large cell lymphoma.<ref name=extranodalmarginalzoneofmucosaassociatedlymphoidtissueMALTlymphoma1>Extranodal marginal zone of mucosa-associated lymphoid tissue (MALT lymphoma). Canadian Cancer Society 2016. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/malt-lymphoma/?region=on. Accessed on January 28, 2016</ref> Complications of MALT lymphoma include [[anemia]], [[cachexia]], [[obstruction]], and [[Perforation|perforation of small intestine]].<ref name=extranodalmarginalzoneofmucosaassociatedlymphoidtissueMALTlymphoma1>Extranodal marginal zone of mucosa-associated lymphoid tissue (MALT lymphoma). Canadian Cancer Society 2016. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/malt-lymphoma/?region=on. Accessed on January 28, 2016</ref> The prognosis is good, and the 10-year survival rate for gastric MALT lymphoma is approximately 90% with a disease-free survival of approximately 70%.<ref name="TroppanWenzl2015">{{cite journal|last1=Troppan|first1=Katharina|last2=Wenzl|first2=Kerstin|last3=Neumeister|first3=Peter|last4=Deutsch|first4=Alexander|title=Molecular Pathogenesis of MALT Lymphoma|journal=Gastroenterology Research and Practice|volume=2015|year=2015|pages=1–10|issn=1687-6121|doi=10.1155/2015/102656}}</ref> However, in rare instances, MALT lymphoma can progress and transform into aggressive high-grade tumors, such as extranodal diffuse large B cell lymphoma (eDLBCL), whereby the 10-year survival rate drops to approximately 42%. | | MALT lymphoma is usually slow growing (indolent), but some can be high grade. If left untreated, MALT lymphoma may progress to develop [[fever]], [[anemia]], [[weight loss]], and large cell lymphoma. Complications of MALT lymphoma include [[anemia]], [[cachexia]], [[obstruction]], and [[Perforation|perforation of small intestine]]. The prognosis is good, and the 10-year survival rate for gastric MALT lymphoma is approximately 90% with a disease-free survival of approximately 70%. However, in rare instances, MALT lymphoma can progress and transform into aggressive high-grade tumors, such as extranodal diffuse large B cell lymphoma (eDLBCL), whereby the 10-year survival rate drops to approximately 42%. |
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| ==Diagnosis== | | ==Diagnosis== |
| ===Staging=== | | ===Staging=== |
| The staging of MALT lymphoma is based on two staging systems: Ann Arbor staging system and the TNM staging system.<ref name=stagingmaltlymphoma1>Staging MALT lymphoma of the stomach. Canadian Cancer Society 2016. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/malt-lymphoma/?region=on. Accessed on January 25, 2016</ref> | | The staging of MALT lymphoma is based on two staging systems: Ann Arbor staging system and the TNM staging system. |
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| ===History and Symptoms=== | | ===History and Symptoms=== |
| When evaluating a patient for MALT lymphoma, you should take a detailed history of the presenting symptom (onset, duration, and progression), other associated symptoms, and a thorough family and past medical history review. Other specific areas of focus when obtaining the history include the review of common associated conditions such as ''[[Helicobacter|H. pyroli]]'' associated gastritis and autoimmune disorders, such as [[Hashimoto's thyroiditis]], [[Sjogren’s syndrome]], and [[Celiac disease]].<ref name=riskfactorsmaltlymphoma1>Risks of Extranodal marginal zone of mucosa-associated lymphoid tissue (MALT lymphoma). Canadian Cancer Society 2016. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/malt-lymphoma/?region=on. Accessed on January 25, 2016</ref><ref name="KinkadeEsan2015">{{cite journal|last1=Kinkade|first1=Zoe|last2=Esan|first2=Olukemi A.|last3=Rosado|first3=Flavia G.|last4=Craig|first4=Michael|last5=Vos|first5=Jeffrey A.|title=Ileal mucosa-associated lymphoid tissue lymphoma presenting with small bowel obstruction: a case report|journal=Diagnostic Pathology|volume=10|issue=1|year=2015|issn=1746-1596|doi=10.1186/s13000-015-0353-6}}</ref> Symptoms of MALT lymphoma depend on the location of the tumor: '''gastric''' and '''non gastric'''.<ref name=sympromaltlymphoma2>Symptoms of MALT lymphoma. Cancer research UK 2016. http://www.cancerresearchuk.org/about-cancer/type/non-hodgkins-lymphoma/about/types/mucosaassociated-lymphoid-tissue-lymphoma. Accessed on January 28, 2016</ref> Symptoms of MALT lymphoma include [[pigastric pain]], [[fatigue]], [[heartburn]], [[fever]], [[cough]] with [[hemoptysis|blood in sputum]], [[dyspnea|shortness of breath]], and/or [[Orbital mass causes|lump in the superior lateral quadrant of the orbit]], [[breast]], [[neck]], or [[salivary gland]].<ref name=sympromaltlymphoma2>Symptoms of MALT lymphoma. Cancer research UK 2016. http://www.cancerresearchuk.org/about-cancer/type/non-hodgkins-lymphoma/about/types/mucosaassociated-lymphoid-tissue-lymphoma. Accessed on January 28, 2016</ref><ref name=sympromaltlymphoma1>Signs and symptoms of gastric lymphoma. Wikipedia 2016. https://en.wikipedia.org/wiki/Gastric_lymphoma. Accessed on January 28, 2016</ref><ref name=nongastricorbitlymphomasym1>Clinical presentation of orbital lymphoma. Dr Craig Hacking and A.Prof Frank Gaillard et al. Radiopaedia 2016. http://radiopaedia.org/articles/orbital-lymphoma. Accessed on January 28, 2016</ref><ref name=sympmalylymmnoingastry>Non-gastric lymphomas – causes, symptoms and treatments. Lymphoma association 2016. https://www.lymphomas.org.uk/sites/default/files/pdfs/Non-Gastric-malt-lymphoma.pdf. Accessed on January 28, 2016</ref> | | When evaluating a patient for MALT lymphoma, detailed history of the presenting symptom (onset, duration, and progression), other associated symptoms, and a thorough family and past medical history should be reviewed. Other specific areas of focus when obtaining the history include the review of common associated conditions such as ''[[Helicobacter pylori|H. pylori]]'' associated [[gastritis]] and [[autoimmune disorders]], such as [[Hashimoto's thyroiditis]], [[Sjogren’s syndrome]], and [[Celiac disease]]. Symptoms of MALT lymphoma depend on the location of the tumor: '''gastric''' and '''non gastric'''. Symptoms of MALT lymphoma include [[epigastric]] pain, [[fatigue]], [[heartburn]], [[fever]], [[cough]] with [[hemoptysis|blood in sputum]], [[dyspnea|shortness of breath]], and/or [[Orbital mass causes|lump in the superior lateral quadrant of the orbit]], [[breast]], [[neck]], or [[salivary gland]]. |
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| ===Physical Examination=== | | ===Physical Examination=== |
| Physical examination findings of MALT lymphoma depend on the location of the tumor: '''gastric''' and '''non gastric'''.<ref name=sympromaltlymphoma2>Symptoms of MALT lymphoma. Cancer research UK 2016. http://www.cancerresearchuk.org/about-cancer/type/non-hodgkins-lymphoma/about/types/mucosaassociated-lymphoid-tissue-lymphoma. Accessed on January 28, 2016</ref> But majority of patients with MALT lymphoma often have no physical findings and lymphadenopathy is uncommon.<ref name="pmid21390139">{{cite journal| author=Ghimire P, Wu GY, Zhu L| title=Primary gastrointestinal lymphoma. | journal=World J Gastroenterol | year= 2011 | volume= 17 | issue= 6 | pages= 697-707 | pmid=21390139 | doi=10.3748/wjg.v17.i6.697 | pmc=PMC3042647 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21390139 }} </ref> | | Physical examination findings of MALT lymphoma depend on the location of the tumor: '''Gastric''' and '''non gastric'''. The majority of patients with MALT lymphoma often have no physical findings and [[lymphadenopathy]] is uncommon. |
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| ===Laboratory Findings=== | | ===Laboratory Findings=== |
| [[Complete blood count]] is performed to check the blood counts.<ref name=sympmalylymmnoingastry>Non-gastric lymphomas – causes, symptoms and treatments. Lymphoma association 2016. https://www.lymphomas.org.uk/sites/default/files/pdfs/Non-Gastric-malt-lymphoma.pdf. Accessed on January 28, 2016</ref> [[Liver function tests]] and [[renal function tests]] are performed to assess the liver and kidneys, respectively.<ref name=sympmalylymmnoingastry>Non-gastric lymphomas – causes, symptoms and treatments. Lymphoma association 2016. https://www.lymphomas.org.uk/sites/default/files/pdfs/Non-Gastric-malt-lymphoma.pdf. Accessed on January 28, 2016</ref> | | [[Complete blood count]] is performed to check the blood counts. [[Liver function tests]] and [[renal function tests]] are performed to assess the liver and kidneys, respectively. |
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| ===Upper GI Endoscopy=== | | ===Upper GI Endoscopy=== |
| The gastric lesion of MALT lymphoma on [[esophagogastroduodenoscopy|upper GI endoscopy]] may look inflammed or ulcerated. Sometimes, a nodular mass, similar to a reactive lymph node, may be visualized.<ref name=sympmalylymmnoingastry>Non-gastric lymphomas – causes, symptoms and treatments. Lymphoma association 2016. https://www.lymphomas.org.uk/sites/default/files/pdfs/Non-Gastric-malt-lymphoma.pdf. Accessed on January 28, 2016</ref><ref name="pmid22943012">{{cite journal| author=Bautista-Quach MA, Ake CD, Chen M, Wang J| title=Gastrointestinal lymphomas: Morphology, immunophenotype and molecular features. | journal=J Gastrointest Oncol | year= 2012 | volume= 3 | issue= 3 | pages= 209-25 | pmid=22943012 | doi=10.3978/j.issn.2078-6891.2012.024 | pmc=PMC3418529 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22943012 }} </ref> | | The gastric lesion of MALT lymphoma on [[esophagogastroduodenoscopy|upper GI endoscopy]] may look [[inflamed]] or ulcerated. Occasionally, a nodular mass, similar to a reactive [[lymph node]], may be visualized. |
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| ===CT=== | | ===CT=== |
| Chest, abdominal, and/or pelvic CT scan is generally performed to evaluate the extent and spread of MALT lymphoma.<ref name="pmid23879202">{{cite journal| author=Nasser QJ, Pfeiffer ML, Romaguera J, Fowler N, Debnam JM, Samaniego F et al.| title=Clinical value of magnetic resonance imaging and other baseline testing for conjunctival mucosa-associated lymphoid tissue lymphoma. | journal=Leuk Lymphoma | year= 2014 | volume= 55 | issue= 5 | pages= 1013-7 | pmid=23879202 | doi=10.3109/10428194.2013.826353 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23879202 }} </ref> If the disease is limited to the stomach (which is assessed with [[computed tomography]]), then 70-80% of patients will have a complete regression on treatment with [[antibiotic]] eradication of ''H. pylori''.<ref name=vujhg>{{cite journal | author = Bayerdörffer E, Neubauer A, Rudolph B, Thiede C, Lehn N, Eidt S, Stolte M | title = Regression of primary gastric lymphoma of mucosa-associated lymphoid tissue type after cure of Helicobacter pylori infection. MALT Lymphoma Study Group. | journal = Lancet | volume = 345 | issue = 8965 | pages = 1591-4 | year = 1995 | id = PMID 7783535}}</ref> | | Chest, abdominal, and/or pelvic CT scan is generally performed to evaluate the extent and spread of MALT lymphoma. If the disease is limited to the stomach (which is assessed with [[computed tomography]]), then 70-80% of patients will have a complete regression on treatment with [[antibiotic]] eradication of ''H. pylori''. |
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| ===MRI=== | | ===MRI=== |
| Chest, abdominal, and/or pelvic CT scan is generally performed to evaluate the extent and spread of MALT lymphoma.<ref name="pmid23879202">{{cite journal| author=Nasser QJ, Pfeiffer ML, Romaguera J, Fowler N, Debnam JM, Samaniego F et al.| title=Clinical value of magnetic resonance imaging and other baseline testing for conjunctival mucosa-associated lymphoid tissue lymphoma. | journal=Leuk Lymphoma | year= 2014 | volume= 55 | issue= 5 | pages= 1013-7 | pmid=23879202 | doi=10.3109/10428194.2013.826353 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23879202 }} </ref> | | Chest, abdominal, and/or pelvic CT scan is generally performed to evaluate the extent and spread of MALT lymphoma. |
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| ===Endoscopic Ultrasound=== | | ===Endoscopic Ultrasound=== |
| Endoscopic ultrasound may be performed to detect the extent of spread of MALT lymphoma through the stomach wall. The endoscopic ultrasound scan may demonstrate the deeper tissues that lie to the stomach lining and also involvement of any adjacent lymph nodes.<ref name=sympmalylymmnoingastry>Non-gastric lymphomas – causes, symptoms and treatments. Lymphoma association 2016. https://www.lymphomas.org.uk/sites/default/files/pdfs/Non-Gastric-malt-lymphoma.pdf. Accessed on January 28, 2016</ref> | | Endoscopic ultrasound may be performed to detect the extent of spread of MALT lymphoma through the stomach wall. The endoscopic ultrasound scan may demonstrate the deeper tissues that lie to the stomach lining and also involvement of any adjacent [[Lymph node|lymph nodes]]. |
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| ===Biopsy=== | | ===Biopsy=== |
| Organ specific endoscopic tissue biopsy is diagnostic of MALT lymphoma.<ref name="pmid16950858">{{cite journal| author=Bacon CM, Du MQ, Dogan A| title=Mucosa-associated lymphoid tissue (MALT) lymphoma: a practical guide for pathologists. | journal=J Clin Pathol | year= 2007 | volume= 60 | issue= 4 | pages= 361-72 | pmid=16950858 | doi=10.1136/jcp.2005.031146 | pmc=PMC2001121 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16950858 }} </ref><ref name=biopsydxmaltlymphoma1>Diagnosis and staging of MALT lymphoma. Wikipedia 2016. https://en.wikipedia.org/wiki/MALT_lymphoma. Accessed on February 1, 2016</ref> To view findings on biopsy characteristic of MALT lymphoma, click [[MALT lymphoma pathophysiology #Microscopic Pathology|'''here''']].<ref name="pmid16950858">{{cite journal| author=Bacon CM, Du MQ, Dogan A| title=Mucosa-associated lymphoid tissue (MALT) lymphoma: a practical guide for pathologists. | journal=J Clin Pathol | year= 2007 | volume= 60 | issue= 4 | pages= 361-72 | pmid=16950858 | doi=10.1136/jcp.2005.031146 | pmc=PMC2001121 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16950858 }} </ref> | | Organ specific endoscopic tissue biopsy is diagnostic of MALT lymphoma. To view findings on biopsy characteristic of MALT lymphoma, click [[MALT lymphoma pathophysiology #Microscopic Pathology|'''here''']]. |
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| ===Other Imaging Findings=== | | ===Other Imaging Findings=== |
| Other imaging studies for MALT lymphoma include barium studies and [[PET scan]]. Barium contrast studies of the esophagus, stomach, small intestine, or the colon may be performed to demonstrate any filling defect in the barium flow which denotes to the presence of any infiltrative mass due to MALT lymphoma.<ref name="pmid16127783">{{cite journal| author=Chung JJ, Kim MJ, Kie JH, Kim KW| title=Mucosa-associated lymphoid tissue lymphoma of the esophagus coexistent with bronchus-associated lymphoid tissue lymphoma of the lung. | journal=Yonsei Med J | year= 2005 | volume= 46 | issue= 4 | pages= 562-6 | pmid=16127783 | doi= | pmc=PMC2815843 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16127783 }} </ref> [[FDG-PET|FDG-PET scan]] may be helpful in diagnosis, staging, and follow-up of MALT lymphoma.<ref name="Beal2005">{{cite journal|last1=Beal|first1=K. P.|title=FDG-PET scanning for detection and staging of extranodal marginal zone lymphomas of the MALT type: a report of 42 cases|journal=Annals of Oncology|volume=16|issue=3|year=2005|pages=473–480|issn=0923-7534|doi=10.1093/annonc/mdi093}}</ref><ref name="pmid17662066">{{cite journal| author=Perry C, Herishanu Y, Metzer U, Bairey O, Ruchlemer R, Trejo L et al.| title=Diagnostic accuracy of PET/CT in patients with extranodal marginal zone MALT lymphoma. | journal=Eur J Haematol | year= 2007 | volume= 79 | issue= 3 | pages= 205-9 | pmid=17662066 | doi=10.1111/j.1600-0609.2007.00895.x | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17662066 }} </ref> | | Other imaging studies for MALT lymphoma include barium studies and [[PET scan]]. Barium contrast studies of the [[esophagus]], stomach, small intestine, or the colon may be performed to demonstrate any filling defect in the barium flow which denotes to the presence of any infiltrative mass due to MALT lymphoma. [[FDG-PET|FDG-PET scan]] may be helpful in diagnosis, staging, and follow-up of MALT lymphoma. |
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| ===Other Diagnostic Studies=== | | ===Other Diagnostic Studies=== |
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| ==Treatment== | | ==Treatment== |
| ===Medical Therapy=== | | ===Medical Therapy=== |
| The optimal therapy for MALT lymphoma depends on the stage at diagnosis. The treatment options for early stage (localized) gastric MALT lymphoma include [[antibiotic therapy]], [[radiotherapy]], [[chemotherapy]], [[surgery]], and [[monoclonal antibodies]].<ref name=treatmentofMALTlymphomaofthestomachrx1>Treatment of MALT lymphoma of the stomach. Canadian Cancer Society 2016. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/malt-lymphoma/?region=on. Accessed on January 28, 2016</ref> The treatment options for advanced stage gastric MALT lymphoma include observation, [[radiotherapy]], [[chemotherapy]], and [[monoclonal antibodies]].<ref name=treatmentofMALTlymphomaofthestomachrx1>Treatment of MALT lymphoma of the stomach. Canadian Cancer Society 2016. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/malt-lymphoma/?region=on. Accessed on January 28, 2016</ref> | | The optimal therapy for MALT lymphoma depends on the stage at diagnosis. The treatment options for early stage (localized) gastric MALT lymphoma include [[antibiotic therapy]], [[radiotherapy]], [[chemotherapy]], [[surgery]], and [[monoclonal antibodies]]. The treatment options for advanced stage gastric MALT lymphoma include observation, [[radiotherapy]], [[chemotherapy]], and [[monoclonal antibodies]]. |
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| ===Surgery=== | | ===Surgery=== |
| [[Surgery]] is not the first-line treatment option for patients with gastric MALT lymphoma. [[gastrectomy|Partial or total gastrectomy]] is usually reserved for patients with early stage gastric MALT lymphoma, if the lymphoma remains after antibiotic therapy or if the gastric lymphoma progresses.<ref name=treatmentofMALTlymphomaofthestomachrx1>Treatment of MALT lymphoma of the stomach. Canadian Cancer Society 2016. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/malt-lymphoma/?region=on. Accessed on January 28, 2016</ref> | | [[Surgery]] is not the first-line treatment option for patients with gastric MALT lymphoma. [[gastrectomy|Partial or total gastrectomy]] is usually reserved for patients with early stage gastric MALT lymphoma, if the lymphoma remains after antibiotic therapy or if the gastric lymphoma progresses. |
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| ===Primary Prevention=== | | ===Primary Prevention=== |
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| ===Secondary Prevention=== | | ===Secondary Prevention=== |
| Secondary prevention strategies following MALT lymphoma include [[urease breath test]], performed 4-6 weeks after completion of the course of antibiotic therapy, and [[endoscopy]] with concurrent [[biopsy]], performed 3-6 months after the treatment is finished.<ref name=sympmalylymmnoingastry>Non-gastric lymphomas – causes, symptoms and treatments. Lymphoma association 2016. https://www.lymphomas.org.uk/sites/default/files/pdfs/Non-Gastric-malt-lymphoma.pdf. Accessed on January 28, 2016</ref> | | Secondary prevention strategies following MALT lymphoma include [[urease breath test]], performed 4-6 weeks after completion of the course of antibiotic therapy, and [[endoscopy]] with concurrent [[biopsy]], performed 3-6 months after the treatment is finished. |
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| ==References== | | ==References== |
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| {{WikiDoc Help Menu}} | | {{WikiDoc Help Menu}} |
| {{WikiDoc Sources}} | | {{WikiDoc Sources}} |
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| | [[Category:Oncology]] |
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| | [[Category:Hematology]] |
| | [[Category:Immunology]] |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sujit Routray, M.D. [2], Ahmed Younes M.B.B.CH [3]
Overview
MALT lymphoma is a common form of non-Hodgkin lymphoma (NHL) involving the mucosa-associated lymphoid tissue (MALT), frequently of the stomach, but virtually any mucosal site can be affected. It is a cancer originating from the B-cells in the marginal zone of the MALT. The evolution of gastric MALT lymphoma is a multistage process starting with the infection of H. pylori resulting in the recruitment of B- and T-cells and other inflammatory cells to the gastric mucosa. Genes involved in the pathogenesis of MALT lymphoma include FOXP1 and BCL6. A characteristic feature of MALT lymphoma is the presence of neoplastic cells within epithelial structures with associated destruction of the glandular architecture to form lymphoepithelial lesions. Most MALT lymphomas occur among individuals of 60 years of age. Males are more commonly affected with gastric MALT lymphoma than females. MALT lymphoma is usually slow growing (indolent), but some can be high grade. If left untreated, MALT lymphoma may progress to develop fever, anemia, weight loss, and large cell lymphoma. Complications of MALT lymphoma include anemia, cachexia, obstruction, and perforation of small intestine. The prognosis is good, and the 10-year survival rate for gastric MALT lymphoma is approximately 90% with a disease-free survival of approximately 70%. However, in rare instances, MALT lymphoma can progress and transform into aggressive high-grade tumors, such as extranodal diffuse large B cell lymphoma (eDLBCL), whereby the 10-year survival rate drops to approximately 42%. The staging of MALT lymphoma is based on two staging systems: Ann Arbor staging system and the TNM staging system. If the disease is limited to the stomach (which is assessed with computed tomography), then 70-80% of patients will have a complete regression on treatment with antibiotic eradication of H. pylori. The optimal therapy for MALT lymphoma depends on the stage at diagnosis. The treatment options for early stage (localized) gastric MALT lymphoma include antibiotic therapy, radiotherapy, chemotherapy, surgery, and monoclonal antibodies. The treatment options for advanced stage gastric MALT lymphoma include observation, radiotherapy, chemotherapy, and monoclonal antibodies. Secondary prevention strategies following MALT lymphoma include urease breath test, performed 4-6 weeks after completion of the course of antibiotic therapy, and endoscopy with concurrent biopsy, performed 3-6 months after the treatment is finished.
Historical Perspective
MALT lymphoma was first described by Isaacson and Wright in 1983.
Classification
There is no classification system established for MALT lymphoma.
Pathophysiology
MALT lymphoma is a form of lymphoma involving the mucosa-associated lymphoid tissue (MALT), frequently of the stomach, but virtually any mucosal site can be afflicted. It is a cancer originating from B cells in the marginal zone of the MALT. The evolution of gastric MALT lymphoma is a multistage process starting with the infection of H. pylori resulting in the recruitment of B- and T-cells and other inflammatory cells to the gastric mucosa. Genes involved in the pathogenesis of MALT lymphoma include FOXP1 and BCL6. Chromosomal translocations are also involved in the pathogenesis of MALT lymphoma, which include t(1;14)(p22;q32), t(11;18)(q21;q21), t(14;18)(q32;q21), and t(3;14)(p14.1;q32). Gastric MALT lymphoma is frequently associated with chronic inflammation as a result of the presence of Helicobacter pylori (72-98%). On microscopic histopathological analysis, MALT lymphoma is characterized by the presence of dense diffuse lymphoid infiltrate of marginal‐zone cells in lamina propria with prominent lymphoepithelial lesions and consisting of small atypical cells with monocytoid features. A characteristic feature of MALT lymphoma is the presence of neoplastic cells within epithelial structures with associated destruction of the glandular architecture to form lymphoepithelial lesions. The neoplastic cells of MALT lymphoma may be positive for B-cell associated antigens (CD19, CD20, CD22, CD79a) that co-express BCL-2, and are negative for CD5, CD10, CD43, and cyclin D1.
Causes
Common causes of gastric MALT lymphoma include H.pyroli infection, long-term immunosuppressant drug therapy, and HIV infection. Common causes of non gastric MALT lymphoma include autoimmune disorders, such as Hashimoto's thyroiditis and Sjogren’s syndrome.
Differentiating MALT Lymphoma from other Diseases
MALT lymphoma must be differentiated from gastric carcinoma, gastritis, diffuse large B cell lymphoma, follicular lymphoma, small cell lymphocytic lymphoma, and mantle cell lymphoma.
Epidemiology and Demographics
MALT lymphoma is the third most common type and accounts for approximately 8% of all cases of non-Hodgkin lymphoma. MALT lymphoma is a disease that tends to affect the elderly population. Most MALT lymphomas occur in people in their 60's. Males are more commonly affected with gastric MALT lymphoma than females. The male to female ratio is approximately 2.43 to 1. There is no racial predilection to MALT lymphoma.
Risk Factors
The most potent risk factor in the development of gastric MALT lymphoma is H.pylori infection. Approximately 5–10% of people with gastric MALT lymphoma, however, do not have an H. pylori infection (H. pylori negative). Other risk factors for gastric MALT lymphoma include long-term immunosuppressant drug therapy and HIV infection. Common risk factors in the development of MALT lymphoma, in areas of the body other than the stomach, are autoimmune disorders such as Hashimoto's thyroiditis, Sjogren’s syndrome, and Celiac disease.
Screening
There is insufficient evidence to recommend routine screening for MALT lymphoma.
Natural History, Complications and Prognosis
MALT lymphoma is usually slow growing (indolent), but some can be high grade. If left untreated, MALT lymphoma may progress to develop fever, anemia, weight loss, and large cell lymphoma. Complications of MALT lymphoma include anemia, cachexia, obstruction, and perforation of small intestine. The prognosis is good, and the 10-year survival rate for gastric MALT lymphoma is approximately 90% with a disease-free survival of approximately 70%. However, in rare instances, MALT lymphoma can progress and transform into aggressive high-grade tumors, such as extranodal diffuse large B cell lymphoma (eDLBCL), whereby the 10-year survival rate drops to approximately 42%.
Diagnosis
Staging
The staging of MALT lymphoma is based on two staging systems: Ann Arbor staging system and the TNM staging system.
History and Symptoms
When evaluating a patient for MALT lymphoma, detailed history of the presenting symptom (onset, duration, and progression), other associated symptoms, and a thorough family and past medical history should be reviewed. Other specific areas of focus when obtaining the history include the review of common associated conditions such as H. pylori associated gastritis and autoimmune disorders, such as Hashimoto's thyroiditis, Sjogren’s syndrome, and Celiac disease. Symptoms of MALT lymphoma depend on the location of the tumor: gastric and non gastric. Symptoms of MALT lymphoma include epigastric pain, fatigue, heartburn, fever, cough with blood in sputum, shortness of breath, and/or lump in the superior lateral quadrant of the orbit, breast, neck, or salivary gland.
Physical Examination
Physical examination findings of MALT lymphoma depend on the location of the tumor: Gastric and non gastric. The majority of patients with MALT lymphoma often have no physical findings and lymphadenopathy is uncommon.
Laboratory Findings
Complete blood count is performed to check the blood counts. Liver function tests and renal function tests are performed to assess the liver and kidneys, respectively.
Upper GI Endoscopy
The gastric lesion of MALT lymphoma on upper GI endoscopy may look inflamed or ulcerated. Occasionally, a nodular mass, similar to a reactive lymph node, may be visualized.
CT
Chest, abdominal, and/or pelvic CT scan is generally performed to evaluate the extent and spread of MALT lymphoma. If the disease is limited to the stomach (which is assessed with computed tomography), then 70-80% of patients will have a complete regression on treatment with antibiotic eradication of H. pylori.
MRI
Chest, abdominal, and/or pelvic CT scan is generally performed to evaluate the extent and spread of MALT lymphoma.
Endoscopic Ultrasound
Endoscopic ultrasound may be performed to detect the extent of spread of MALT lymphoma through the stomach wall. The endoscopic ultrasound scan may demonstrate the deeper tissues that lie to the stomach lining and also involvement of any adjacent lymph nodes.
Biopsy
Organ specific endoscopic tissue biopsy is diagnostic of MALT lymphoma. To view findings on biopsy characteristic of MALT lymphoma, click here.
Other Imaging Findings
Other imaging studies for MALT lymphoma include barium studies and PET scan. Barium contrast studies of the esophagus, stomach, small intestine, or the colon may be performed to demonstrate any filling defect in the barium flow which denotes to the presence of any infiltrative mass due to MALT lymphoma. FDG-PET scan may be helpful in diagnosis, staging, and follow-up of MALT lymphoma.
Other Diagnostic Studies
There are no other diagnostic studies associated with MALT lymphoma.
Treatment
Medical Therapy
The optimal therapy for MALT lymphoma depends on the stage at diagnosis. The treatment options for early stage (localized) gastric MALT lymphoma include antibiotic therapy, radiotherapy, chemotherapy, surgery, and monoclonal antibodies. The treatment options for advanced stage gastric MALT lymphoma include observation, radiotherapy, chemotherapy, and monoclonal antibodies.
Surgery
Surgery is not the first-line treatment option for patients with gastric MALT lymphoma. Partial or total gastrectomy is usually reserved for patients with early stage gastric MALT lymphoma, if the lymphoma remains after antibiotic therapy or if the gastric lymphoma progresses.
Primary Prevention
There are no primary preventive measures available for MALT lymphoma.
Secondary Prevention
Secondary prevention strategies following MALT lymphoma include urease breath test, performed 4-6 weeks after completion of the course of antibiotic therapy, and endoscopy with concurrent biopsy, performed 3-6 months after the treatment is finished.
References
Template:WikiDoc Sources