Increased Incidence of Follicular Lymphoma in the Duodenum

Abstract

The incidence of indolent lymphomas in the lymph nodes and extranodal regions is quite different. Follicular lymphoma (FL) is most common in the nodes, and it seems to be least common in the gastrointestinal (GI) tract, where mucosa-associated lymphoid tissue lymphoma arises most frequently. The authors report that the incidence of FL is unexpectedly high in the duodenum compared with other portions of the GI tract. FL was detected in only eight of 222 cases of GI lymphoma (3.6%). However, five cases of FL arose in the duodenum, which accounted for 38.5% of 13 duodenal lymphomas. Only in two patients did FL arise in either the stomach or the colorectum, and in the remaining patients FL was widespread with lymphomatous polyposis. Duodenal FL was composed of neoplastic follicles with small cleaved cells in dominance, and the immunophenotype of the lymphoma cells was CD10+, BCL-2+, CD20+, CD75+, CD79+, CD3-, CD5-, cyclin D1-, CD23-, and CD45RO-. All the patients were women age 37 to 66 years (average age, 52.4 yrs). In all patients the lymphoma was present around the ampulla of Vater, and four of five patients showed multiple small-size polyps. Although lymphoma cell infiltration was confined to the submucosa in the four patients examined, the regional lymph nodes were involved partially in two patients without distant metastasis. All patients are alive at 2 to 50 months of follow up (average, 27 mos), which is comparable with the prognosis for indolent nodal lymphomas. These results suggest that the duodenum has a distinct background of histogenesis of the lymphomas and that biopsy specimens from the duodenum with multiple polyps should be examined carefully.

Translocation t(14;18)/IGH‐BCL2 in gastrointestinal follicular lymphoma

“…predominance of duodenal involvement of FL is in agreement with most previous studies, 3,6,7,9,14 However, LeBrun et al 21 and Damaj et al 15 reported that the ileum was the most frequent site. In recent studies that evaluated an extensive area of the small intestine by DBE, involvement of the jejunum and/or the ileum was identified in 80% to 100% of patients.…”

“…[2][3][4] Recently, balloon-assisted enteroscopies-that is, double-balloon endoscopy (DBE) and single-balloon endoscopy (SBE)-have enabled the examination of an extensive area of the small bowel and the detection of diminutive small intestinal lesions. The usefulness of DBE for the practical diagnosis of intestinal FL has been reported in several publications.…”

Molecular pathways in follicular lymphoma

“…These rare FLs harbor the t(14;18) involving IgH/BCL-2 as determined by polymerase chain reaction, evolve in the gut mucosa, express the mucosal homing integrin a4b7, typically express IgA and have a low tendency to spread systemically and by consequence have a good clinical prognosis. [4][5][6]83 These features indicate that GI-FLs originate from local, antigen-responsive precursor cells and their sessile nature suggests continued dependence on Ags, for example, originating from the gut lumen, also during the tumor stage. 4 It has been reported that in 55 of 79 FLs analyzed (79%), replacement mutations in IgV H result in generation of aminoacid motifs being potential N-glycosylation sites.…”

Duodenal and nodal follicular lymphomas are distinct: the former lacks activation-induced cytidine deaminase and follicular dendritic cells despite ongoing somatic hypermutations

“…1 Although they express CD10 and bcl-2 and harbor t(14;18) translocation similar to nodal follicular lymphomas, 2 most of them are obviously at lower clinical stages than the nodal cases (most are stage I-II) and have lower histological grading with rather dominantly uniform intermediate small cleaved cells.…”