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Extranodal Marginal Zone Lymphoma of Mucosa-Associated Lymphoid Tissue Involving the Digestive System

MedGen UID:
1763537
•Concept ID:
C5419894
•
Neoplastic Process

Definition

An extranodal low-grade B-cell non-Hodgkin lymphoma arising in mucosal or glandular tissues of the digestive system. It recapitulates the cytoarchitectural features of mucosa-associated lymphoid tissue (MALT). It is composed of small lymphoid cells, often including marginal zone cells. (WHO 2019) [from NCI]

Professional guidelines

PubMed

Radiation treatment planning techniques for lymphoma of the stomach.
Della Biancia C, Hunt M, Furhang E, Wu E, Yahalom J
Int J Radiat Oncol Biol Phys 2005 Jul 1;62(3):745-51. doi: 10.1016/j.ijrobp.2004.10.025. PMID: 15936555
Gastric low-grade B-cell MALT lymphoma: treatment, response, and genetic alteration.
Nakamura T, Inagaki H, Seto M, Nakamura S
J Gastroenterol 2003;38(10):921-9. doi: 10.1007/s00535-003-1234-4. PMID: 14614598
Gastric MALT lymphoma: from aetiology to treatment.
Du MQ, Isaccson PG
Lancet Oncol 2002 Feb;3(2):97-104. doi: 10.1016/s1470-2045(02)00651-4. PMID: 11902529

Recent clinical studies

Etiology

Lymphomas of the salivary glands: a systematic review.
Al-Khafaf AE, Al-Shahrestani F, Baysal Y, Rahbek Gjerdrum LM, Heegaard S, Pedersen LM, Homøe P
Acta Otolaryngol 2023 Jul-Aug;143(7):610-616. Epub 2023 Aug 12 doi: 10.1080/00016489.2023.2226689. PMID: 37572309
Update on lymphoproliferative disorders of the gastrointestinal tract: disease spectrum from indolent lymphoproliferations to aggressive lymphomas.
Montes-Moreno S, King RL, Oschlies I, Ponzoni M, Goodlad JR, Dotlic S, Traverse-Glehen A, Ott G, Ferry JA, Calaminici M
Virchows Arch 2020 May;476(5):667-681. Epub 2019 Nov 26 doi: 10.1007/s00428-019-02704-8. PMID: 31773249
TREX1 variants in Sjogren's syndrome related lymphomagenesis.
Nezos A, Makri P, Gandolfo S, De Vita S, Voulgarelis M, Crow MK, Mavragani CP
Cytokine 2020 Aug;132:154781. Epub 2019 Jul 18 doi: 10.1016/j.cyto.2019.154781. PMID: 31326279
Prognostic markers of lymphoma development in primary Sjögren syndrome.
Retamozo S, Brito-Zerón P, Ramos-Casals M
Lupus 2019 Jul;28(8):923-936. Epub 2019 Jun 19 doi: 10.1177/0961203319857132. PMID: 31215845
Role of Helicobacter pylori in gastric mucosa-associated lymphoid tissue lymphomas.
Pereira MI, Medeiros JA
World J Gastroenterol 2014 Jan 21;20(3):684-98. doi: 10.3748/wjg.v20.i3.684. PMID: 24574742Free PMC Article

Diagnosis

Extranodal MALT Lymphoma in the Oral Cavity: A Series of Three Cases with Review of Literature.
Kaur R, Shetty D, Bagal BP, Gujral S, Sengar M, Laskar S, Nayak L, Shet T
Head Neck Pathol 2022 Dec;16(4):1242-1250. Epub 2022 Jun 8 doi: 10.1007/s12105-022-01461-6. PMID: 35674932Free PMC Article
Prognostic markers of lymphoma development in primary Sjögren syndrome.
Retamozo S, Brito-Zerón P, Ramos-Casals M
Lupus 2019 Jul;28(8):923-936. Epub 2019 Jun 19 doi: 10.1177/0961203319857132. PMID: 31215845
Non-Hodgkin's lymphoma of the oral cavity and maxillofacial region: a pathologist viewpoint.
Abdelwahed Hussein MR
Expert Rev Hematol 2018 Sep;11(9):737-748. Epub 2018 Aug 21 doi: 10.1080/17474086.2018.1506326. PMID: 30058399
Lymphoproliferative Disorders of the Gastrointestinal Tract.
Skinnider BF
Arch Pathol Lab Med 2018 Jan;142(1):44-52. Epub 2017 Aug 22 doi: 10.5858/arpa.2016-0610-RA. PMID: 28829152
Helicobacter pylori infection in gastric mucosa-associated lymphoid tissue lymphoma.
Park JB, Koo JS
World J Gastroenterol 2014 Mar 21;20(11):2751-9. doi: 10.3748/wjg.v20.i11.2751. PMID: 24659867Free PMC Article

Therapy

Outcomes After Reduced-Dose Intensity Modulated Radiation Therapy for Gastric Mucosa-Associated Lymphoid Tissue (MALT) Lymphoma.
Pinnix CC, Gunther JR, Milgrom SA, Cruz Chamorro RJ, Medeiros LJ, Khoury JD, Amini B, Neelapu S, Lee HJ, Westin J, Fowler N, Nastoupil L, Dabaja B
Int J Radiat Oncol Biol Phys 2019 Jun 1;104(2):447-455. Epub 2019 Feb 12 doi: 10.1016/j.ijrobp.2019.02.002. PMID: 30769175Free PMC Article
Prevalence and Implications of Bone Marrow Involvement in Patients with Gastric Mucosa-Associated Lymphoid Tissue Lymphoma.
Choi SI, Kook MC, Hwang S, Kim YI, Lee JY, Kim CG, Choi IJ, Lee H, Eom HS, Cho SJ
Gut Liver 2018 May 15;12(3):278-287. doi: 10.5009/gnl17217. PMID: 29409307Free PMC Article
Primary mucosa-associated lymphoid tissue lymphoma of the salivary glands: a multicenter Rare Cancer Network study.
Anacak Y, Miller RC, Constantinou N, Mamusa AM, Epelbaum R, Li Y, Calduch AL, Kowalczyk A, Weber DC, Kadish SP, Bese N, Poortmans P, Kamer S, Ozsahin M
Int J Radiat Oncol Biol Phys 2012 Jan 1;82(1):315-20. Epub 2010 Nov 13 doi: 10.1016/j.ijrobp.2010.09.046. PMID: 21075560
Helicobacter pylori infection and gastric MALTomas: an up-to-date and therapy highlight.
Santacroce L, Cagiano R, Del Prete R, Bottalico L, Sabatini R, Carlaio RG, Prejbeanu R, Vermesan H, Dragulescu SI, Vermesan D, Motoc A, Losacco T
Clin Ter 2008 Nov-Dec;159(6):457-62. PMID: 19169609
Helicobacter pylori eradication and its implications for the future.
Blaser MJ
Aliment Pharmacol Ther 1997 Apr;11 Suppl 1:103-7. doi: 10.1046/j.1365-2036.11.s1.7.x. PMID: 9146796

Prognosis

Predicting lymphoma development in patients with Sjögren's syndrome.
De Vita S, Gandolfo S
Expert Rev Clin Immunol 2019 Sep;15(9):929-938. Epub 2019 Sep 12 doi: 10.1080/1744666X.2019.1649596. PMID: 31347413
Prognostic markers of lymphoma development in primary Sjögren syndrome.
Retamozo S, Brito-Zerón P, Ramos-Casals M
Lupus 2019 Jul;28(8):923-936. Epub 2019 Jun 19 doi: 10.1177/0961203319857132. PMID: 31215845
Non-Hodgkin's lymphoma of the oral cavity and maxillofacial region: a pathologist viewpoint.
Abdelwahed Hussein MR
Expert Rev Hematol 2018 Sep;11(9):737-748. Epub 2018 Aug 21 doi: 10.1080/17474086.2018.1506326. PMID: 30058399
Helicobacter pylori infection in gastric mucosa-associated lymphoid tissue lymphoma.
Park JB, Koo JS
World J Gastroenterol 2014 Mar 21;20(11):2751-9. doi: 10.3748/wjg.v20.i11.2751. PMID: 24659867Free PMC Article
Role of Helicobacter pylori in gastric mucosa-associated lymphoid tissue lymphomas.
Pereira MI, Medeiros JA
World J Gastroenterol 2014 Jan 21;20(3):684-98. doi: 10.3748/wjg.v20.i3.684. PMID: 24574742Free PMC Article

Clinical prediction guides

Extranodal MALT Lymphoma in the Oral Cavity: A Series of Three Cases with Review of Literature.
Kaur R, Shetty D, Bagal BP, Gujral S, Sengar M, Laskar S, Nayak L, Shet T
Head Neck Pathol 2022 Dec;16(4):1242-1250. Epub 2022 Jun 8 doi: 10.1007/s12105-022-01461-6. PMID: 35674932Free PMC Article
Clinical picture, outcome and predictive factors of lymphoma in primary Sjögren's syndrome: results from a harmonized dataset (1981-2021).
Chatzis LG, Stergiou IE, Goules AV, Pezoulas V, Tsourouflis G, Fotiadis D, Tzioufas AG, Voulgarelis M
Rheumatology (Oxford) 2022 Aug 30;61(9):3576-3585. doi: 10.1093/rheumatology/keab939. PMID: 34940812
Predicting lymphoma development in patients with Sjögren's syndrome.
De Vita S, Gandolfo S
Expert Rev Clin Immunol 2019 Sep;15(9):929-938. Epub 2019 Sep 12 doi: 10.1080/1744666X.2019.1649596. PMID: 31347413
Helicobacter pylori infection in gastric mucosa-associated lymphoid tissue lymphoma.
Park JB, Koo JS
World J Gastroenterol 2014 Mar 21;20(11):2751-9. doi: 10.3748/wjg.v20.i11.2751. PMID: 24659867Free PMC Article
Advances in salivary gland pathology.
Cheuk W, Chan JK
Histopathology 2007 Jul;51(1):1-20. Epub 2007 May 31 doi: 10.1111/j.1365-2559.2007.02719.x. PMID: 17539914

Recent systematic reviews

Lymphomas of the salivary glands: a systematic review.
Al-Khafaf AE, Al-Shahrestani F, Baysal Y, Rahbek Gjerdrum LM, Heegaard S, Pedersen LM, Homøe P
Acta Otolaryngol 2023 Jul-Aug;143(7):610-616. Epub 2023 Aug 12 doi: 10.1080/00016489.2023.2226689. PMID: 37572309

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