Primary extranodal lymphomas of stomach: Clinical presentation, diagnostic pitfalls and management

Second Department of Internal Medicine Propaedeutic, Athens University Medical School, University General Hospital Attikon, Haidari, Greece.
Annals of Oncology (Impact Factor: 7.04). 12/2008; 19(12):1992-9. DOI: 10.1093/annonc/mdn525
Source: PubMed


Gastrointestinal lymphoma is the most common form of extranodal lymphoma, accounting for 30%–40% of cases. The most commonly
involved site is the stomach (60%–75% of cases), followed by the small bowel, ileum, cecum, colon and rectum. The most common
histological subtypes are diffuse large B-cell lymphoma (DLBCL) and marginal zone B-cell lymphoma of the mucosa-associated
lymphoid tissue (MALT). Helicobacter pylori infection has been implicated in the pathogenesis of MALT gastric lymphoma, but its role in gastric diffuse large B-cell
non-Hodgkin's lymphoma (NHL) is controversial. The therapeutic approach for patients with gastric NHL has been revised over
the last 10 years. Conservative treatment with anthracycline-based chemotherapy alone or in combination with involved-field
radiotherapy has replaced gastrectomy as standard therapy in cases with DLBCL. Additionally, MALT lymphomas are mainly treated
with antibiotics alone, which can induce lasting remissions in those cases associated with H. pylori infection. Nevertheless, various therapeutic aspects for primary gastric lymphomas are still controversial and several questions
remain unanswered. Among others, the role of rituximab, consolidation radiotherapy as well as H. pylori eradication in histological aggressive subtypes warrants better clarification.

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    • "Gastric DLBCL may be treated with surgical resection, chemotherapy and radiotherapy either alone or in combination. Surgical intervention may be reserved for treating complications such as major bleeding, obstruction or perforation (2). Conservative treatment with chemotherapy with or without radiotherapy is the preferred treatment method (3,4). "
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    • "Virtually all gastric lymphomas arise from B-cell [2]; T-cell neoplasia of stomach is extremely rare [5]. They include marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT), which account for nearly 50% of gastric lymphomas, and diffuse large B-cell lymphomas (DLBCL), whilst both follicular and mantle cell lymphomas are infrequent [5,6]. The marginal zone lymphoma – usually named MALT lymphoma – is typically a low-grade neoplasia, characterized by a dense lymphoid infiltrate mainly composed of small-size lymphocytes that invade and destroy gastric glands, configuring the so-called ‘lymphoepithelial lesion’ which is pathognomonic of lymphoma [7]. "
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    ABSTRACT: The stomach is the most frequent site of extranodal lymphoma. Gastric lymphoma originating from mucosa-associated lymphoid tissue (MALT) is typically a low-grade, B-cell neoplasia strongly associated with Helicobacter pylori (H. pylori) infection. Only certain H. pylori strains in some predisposed patients determine lymphoma development in the stomach, according to a strain-host-organ specific process. The clinical presentation is poorly specific, symptoms ranging from vague dyspepsia to alarm symptoms. Similarly, different endoscopy patterns have been described for gastric lymphoma. H. pylori eradication is advised as first-line therapy in early stage disease, and complete lymphoma remission is achieved in 75% of cases. Neoplasia stage, depth of infiltration in the gastric wall, presence of the API2-MALT1 translocation, localization in the stomach, and patient ethnicity have been identified as predictors of remission. Recent data suggests that H. pylori eradication therapy may be successful for gastric lymphoma treatment also in a small subgroup (15%) of H. pylori-negative patients. The overall 5-year survival and disease-free survival rates are as high as 90% and 75%, respectively. Management of patients who failed to achieve lymphoma remission following H. pylori eradication include radiotherapy, chemotherapy and, in selected cases, surgery.
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    • "The use of different staging systems for intestinal DLBCL hampers comparisons of data in the literature (4, 23, 24). Moreover, the definitions of limited disease and of extended/advanced disease vary among the authors (15, 24). "
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