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Choi and Kim: Diagnosis and Treatment of Gastric MALT Lymphoma
Review Article

Korean J Gastroenterol 2011;57(5):272-280.

Published online: 4 October 2011

DOI: https://doi.org/10.4166/kjg.2011.57.5.272

Diagnosis and Treatment of Gastric MALT Lymphoma

Mun Ki Choi, Gwang Ha Kim

Department of Internal Medicine, Pusan National University School of Medicine, Busan, Korea

Correspondence to: Gwang Ha Kim, Department of Internal Medicine, Pusan National University School of Medicine, 1-10, Ami-dong, Seo-gu, Busan 602-739, Korea. Tel: +82-51-240-7869, Fax: +82-51-244-8180, E-mail: doc0224@pusan.ac.kr

Copyright © 2011 Korean Ophthalmological Society

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Gastric mucosa-associated lymphoid tissue (MALT) lymphoma represents approximately 40% of gastric lymphomas, and its incidence is increasing. An early diagnosis for gastric MALT lymphoma is important, but not easy due to non-specific symptoms and endoscopic findings. Diagnosis is based on the histopathologic evaluation of multiple, deep and repeated biopsies taken from normal and any abnormal appearing sites of the stomach. In addition, the presence of Helicobacter pylori (H. pylori) infection must be determined to determine therapeutic approach. Endoscopic ultrasonography (EUS) is essential for the evaluation of regional lymph nodes and the depth of tumor invasion in the gastric wall, for predicting response to H. pylori eradication, and for monitoring tumor regression or recurrence. The eradication of H. pylori is recommended as an initial treatment for low-grade gastric MALT lymphoma with H. pylori infection. Both radiation therapy and chemotherapy are suitable alternative options for H. pylori-negative, refractory, or high-grade gastric MALT lymphoma. But, the role of surgery is diminishing. After treatment, strict endoscopic regular follow-up including EUS is recommended with multiple biopsies. However, controversy remains regarding the best diagnosis, treatment and follow-up strategy for this disease.

Keywords: Gastric MALT lymphoma, Diagnosis, Treatment

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Fig. 1.
Endoscopic findings of superficial type gastric MALT lymphoma. (A) IIc-like type. (B) Submucosal tumor type. (C) Multiple erosion type.(D) Cobblestone-mucosa type. (E) Partial-fold-thickening type. (F) Discoloration type.
kjg-57-272f1.tif
Fig. 2.
Histological features of gastric MALT lymphoma. Neoplastic lymphoid cells invaded gastric glands (lymphoepithelial lesion, arrow).
kjg-57-272f2.tif
Fig. 3.
Algorithm suggested for the diagnostic procedure of gastric biopsies.15*Lymphoma of MALT type.
kjg-57-272f3.tif
Fig. 4.
EUS findings of gastric MALT lymphoma. (A) Tumor limited to the mucosa (T1). (B) Tumor limited to the submucosa (T2). (C) Tumor extended to the serosa (T3).
kjg-57-272f4.tif
Fig. 5.
Pre- and post-treatment endoscopic findings of gastric MALT lymphoma after H. pylori eradication. (A-C) Pre-treatment endoscopic findings. (D-F) Post-treatment endoscopic findings. Whitish discolored areas appeared at the site where the previous MALT lymphoma disappeared after H. pylori eradication.
kjg-57-272f5.tif
Table 1.
Low-grade Gastric MALT Lymphoma: Most Common Clinical Features6
Age (median, year) 63
Male:Female ratio 1:1
Localized disease (stage I) 88%
Bone marrow infiltration 7%
Main symptoms at presentation  
Epigastric or abdominal pain 53%
Dyspepsia 32%
Nausea and vomiting 8%
Gastric bleeding 2%
B symptoms 1%
Elevated LDH 1%
Elevated β2-microglobulin 4%
Main endoscopic findings  
Erythema 30%
Erosions 23%
Ulcers 47%
Gastric localization  
Antrum 41%
Body 12%
Fundus 11%
Multifocal 33%
Gastric stump 3%
Table 2.
Differential Points of Endoscopic Findings between EGC IIc and MALT Lymphoma
  EGC IIc MALT lymphoma
Lesion    
Gloss Decreased Preserved
Cobblestone appearance Few Many
Submucosal tumor-like component Few Often
Border    
Distinctness/Continuity Distinct/Continuity (＋) Indistinct/Continuity (−)
Mucosal folds Clubbing, abrupt cut-off Convex or concave tapering
Moth-eaten appearance Present Absent
Number of lesions Single Single to multiple

EGC, early gastric cancer.

Table 3.
Histologic Scoring of Lymphoid Infiltrations in the Stomach5
Score Diagnosis Histological features
0 Normal Scattered plasma cells in lamina propria. No lymphoid follicles.
1 Chronic active gastritis Small clusters of lymphocytes in lamina propria. No lymphoid follicle. No lymphoepithelial lesions.
2 Chronic active gastritis with florid lymphoid follicle formation Prominent lymphoid follicles with surrounding mantle zone and plasma cells. No lymphoepithelial lesions.
3 Suspicious lymphoid infiltrate, probably reactive Lymphoid follicles surrounded by small lymphocytes that infiltrate diffusely in lamina propria and occasionally into epithelium.
4 Suspicious lymphoid infiltrate, probably lymphoma Lymphoid follicles surrounded by marginal zone cells that infiltrate diffusely in lamina propria and into epithelium in small groups.
5 MALT lymphoma Presence of dense infiltrate of marginal zone cells in lamina propria with prominent lymphoepithelial lesions.
Table 4.
EUS Staging of Gastric MALT Lymphoma
T1a Superficial mucosa (includes the 1 st hyperechoic layer)
T1b Deeper mucosa to muscularis mucosa (includes up to the 2nd hypoechoic layer)
T2 To submucosa (includes the 3 rd hyperechoic layer)
T3 Beyond submucosa (includes the 4 th layer corresponding to the muscularis propria and the 5th layer corresponding to the serosa)
Table 5.
Ann Arbor Classification of Extranodal Lymphoma (Modified by Musshoff)23
I E: Lymphoma restricted to GI tract on one side of diaphragm
I E1: Infiltration limited to mucosa and submucosa
I E2: Lymphoma extending beyond submucosa
II E: Lymphoma additionally infiltrating LNs on same side of diaphragm
II E1: Infiltration of regional LNs
II E2: Infiltration of LNs beyond regional LNs
III E: Lymphoma infiltrating GI tract and/or LNs on both sides of diaphragm
IV E: Localized infiltration of associated LNs together with diffuse/disseminated extra-GI organs involvement

GI, gastrointestinal; LN, lymph node.

Table 6.
Recommended Staging Procedures for Gastric Lymphoma6
History (duration and presence of local or systemic symptoms)
Physical examination (careful evaluation of all lymph node regions, inspection of the upper airways and tonsils, clinical evaluation of the size of liver and spleen, detection of any palpable mass)
Laboratory tests, including complete blood counts and peripheral blood smear, LDH & β2-microglobulin levels, evaluation of renal and liver function
Bone marrow biopsy
Standard posteroanterior and lateral chest radiographs
Abdominal and pelvic computed tomography scan
Gastroduodenal endoscopy with multiple gastric biopsies from all the visible lesions and the noninvolved areas with a complete mapping of the organ
Gastric endoscopic ultrasonography
Table 7.
Suggested Therapeutic Algorithm for Gastric MALT Lymphoma
Stage H. pylor First-line treatment i Second-line treatment
I E1 (＋) H. pylori eradication Radiation
  (−) Radiation Chemotherapy (or surgery)
I E2/II E1 (＋) Radiation＋ H. pylori eradication Chemotherapy
E2 E1 (−) Radiation Chemotherapy
II E2/III E/IV E   Chemotherapy ± H. pylori eradication
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