Gastrointestinal follicular lymphoma: review of the literature

Abstract

Gastrointestinal follicular lymphoma (GI-FL) is a relatively rare disease, accounting for only 1%-3.6% of gastrointestinal non-Hodgkin's lymphoma. Although the duodenum and terminal ileum are considered to be the most common sites of origin, the development of wireless capsule endoscopy and double-balloon enteroscopy has increased the detection of GI-FL in every part of the small intestine. Approximately 70% of patients with GI-FL are estimated to have multiple lesions throughout the entire gastrointestinal tract. FL is a low-grade lymphoma that usually develops very slowly. If the lymphoma causes no symptoms, immediate treatment may not be necessary. Standard therapy has not yet been established for GI-FL, but chemotherapy, radiotherapy, monoclonal antibody therapy, or a combination of these therapies, is sometimes performed based on the therapeutic regimens for nodal FL. Regimens including conventional chemotherapy with rituximab, which achieve high response rates in nodal FL, are commonly used for GI-FL. The long-term clinical outcome of GI-FL is unclear. The results of a few series on the long-term outcomes of patients with GI-FL treated with conventional therapy indicate a median relapse-free time ranging from 31 to 45 months. On the other hand, in patients with GI-FL who were followed without treatment, the median time to disease progression was 37.5 months. Thus, whether to initiate aggressive therapy or whether to continue watchful waiting in patients with GI-FL is a critically important decision. Ongoing research on biomarkers to guide individualized GI-FL therapy may provide invaluable information that will lead to the establishment of a standard therapeutic regimen.

Follicular lymphoma in young adults: a clinicopathological and molecular study of 200 patients

“…17 Three of our cases were intestinal follicular lymphoma-two in the duodenum and one in the stomach-all three achieved complete remission after treatment with no documented spread to other sites. Lobetti-Bodoni et al 18 studied 153 follicular lymphoma patients o40 years in comparison with 850 patients 440 years, and found that younger patients had longer overall survival and longer cause-specific survival.…”

Flow Cytometric Analysis of Kappa and Lambda Light Chain Expression in Endoscopic Biopsy Specimens before the Diagnosis of B-Cell Lymphoma

“…18 In Japan, GI tract lesions are usually examined by GI endoscopy with biopsy as routine practice. 6 To our knowledge, there are only a few reports of the flow cytometric analysis of endoscopic biopsy specimens for the diagnosis of BCL. 7,19,20 FCM is accurate and quantitative.…”

“…4,5 If GI tract lesions are suspected, gastrointestinal endoscopy with biopsy is performed as routine practice in Japan. 6 Previously, we reported that FCM was useful for evaluating B-cell clonality in endoscopic biopsy specimens from 10 patients with GI tract BCL. 7 We extended our work to confirm these results in a large number of patients with GI tract BCL.…”

Extranodal and splenic small B-cell lymphoma

“…In a recent review of 249 cases (244 from the literature and 5 new cases) of GI follicular lymphoma, 41 it was noted that the demographics were similar to nodal follicular lymphoma (M:F 1:1; median age 56 years). The majority occurred in the duodenum (usually as polyps rather than obstructing lesions) or small intestine and in contrast to nodal follicular lymphoma, the majority of GI follicular lymphomas present in clinical stages I/II (93.2%).…”

“…41,42 Morphologically the infiltrate consists of either a single abnormal follicle or small groups of neoplastic follicles, which may be limited to the mucosa or extend more deeply through the wall (Figure 2). The immunophenotype of GI follicular lymphoma is identical to the nodal counterpart with expression of pan-B-cell markers, CD10 and BCL6 (Figure 2).…”