Primary diffuse large B-cell lymphoma of the stomach

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Abstract

The stomach is the extranodal site most commonly involved by non-Hodgkin lymphomas. Diffuse large B-cell lymphoma is the most common histotype category arising in this organ. This is an aggressive lymphoma usually presenting as limited disease, being associated or not to Helicobacter pylori infection and mucosa-associated lymphoid tissue-type areas. Histopathological characteristics are similar to those reported for other diffuse large B-cell lymphomas. It occurs more frequently in males with a median age ranging between 50 and 60 years. With an adequate therapeutic strategy, its prognosis is good, with a 5-year overall survival near to 90%. Conservative treatment with anthracycline-containing chemotherapy, followed or not by involved-field radiotherapy has replaced gastrectomy as standard approach against this malignancy. Several questions on the best treatment remain unanswered. Among others, the role of rituximab, consolidation radiotherapy as well as of more conservative approaches like H. pylori-eradicating antibiotic therapy should be better defined.

Section snippets

Incidence and risk factors

Primary non-Hodgkin lymphomas (NHL) of the gastrointestinal tract represent 3–4% of all malignancies arising in the digestive tract. The most commonly involved site is the stomach (60% of cases), followed by small bowel, ileum, caecum, colon, and rectum [1]. Primary gastric NHL is a malignancy localised in the stomach, with or without perigastric and/or abdominal lymph nodal involvement, and constitutes 20–30% of all extranodal NHLs. Any lymphoma category can arise in the stomach, but over 70%

Pathology

PGDLCL is defined as a malignancy composed of large cells (nuclei at least twice the size of a small lymphocyte; usually larger than tissue macrophage nuclei) with vesicular nuclei, prominent nucleoli, basophilic cytoplasm and a moderate to high proliferation fraction. This malignancy constitutes 40–70% of all gastric lymphomas. Lesions are characterized by an intense cellular infiltration of the lamina propria. In most cases, the predominant cells resemble either centroblasts (large noncleaved

Clinical presentation and behavior

PGDLCL occurs more frequently in males, with a median age range of occurrence of 50–60 years [1], [13], [14]. Clinical presentation is similar to that of gastric cancer or of benign ulcer. The majority of patients report epigastric pain (70% of cases) or dyspepsia (30%). The duration of symptoms before diagnosis is variable, ranging between a few weeks to several years. Weight loss is observed in 40% of patients, which is usually a consequence of the dyspepsia; only infrequently is it a

Staging

Complete staging work-up for PGDLCL includes an accurate physical examination (Waldeyer's ring involvement should be excluded) complete haematological and biochemical exams, total-body computerized tomography, gastrointestinal tract examination, and bone marrow aspiration and biopsy. In the past, abdominal staging was performed by exploratory laparotomy, and gastrectomy was considered as relevant in primary treatment. In the last few decades, however, a surgical approach has increasingly been

Prognostic factors

No reliable prognostic factors in PGDLCL have been definitively established. This may be due to the fact that studies have involved heterogeneous management and have been retrospective in nature, and the fact that, in several cases, patients with gastric lymphomas have been analyzed together with patients who have intestinal lymphomas. Those series usually included patients with all stages of disease. In the largest series of gastrointestinal lymphoma, bulky lesion, stage, histology,

Treatment

There is a growing consensus that conservative combined treatment with primary conventional-dose anthracycline-containing chemotherapy, which may or may not be followed by radiation therapy, is suitable for individual clinical use on a type C basis for patients with stage I–II diffuse large B-cell lymphoma of the stomach. Surgery, which until a few decades ago was deemed an essential first-line therapeutic intervention, is now relegated to patients with massive bleeding or perforation at

Andrés J.M. Ferreri M.D. is Coordinator of the Lymphoma Unit and Vice Director of the Medical Oncology Unit, Department of Oncology, San Raffaele Scientific Institute, Milan, Italy.

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    Andrés J.M. Ferreri M.D. is Coordinator of the Lymphoma Unit and Vice Director of the Medical Oncology Unit, Department of Oncology, San Raffaele Scientific Institute, Milan, Italy.

    Carlos Montalbán Sanz M.D., Ph.D. is Head of the Section Oncology & Lymphoproliferative Diseases, Department of Internal Medicine, Hospital Ramón y Cajal, Madrid, Spain; Associate Professor, Department of Medicine, Universidad de Alcalá de Henares, Spain.

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