Primary gastrointestinal follicular lymphoma involving the duodenal second portion is a distinct entity: A multicenter, retrospective analysis in Japan

Abstract

We conducted a multicenter, retrospective study to determine the anatomical distribution and prognostic factors of gastrointestinal (GI) follicular lymphoma (FL). This study included 125 patients with stage I and II 1 GI-FL. Of the 125 patients, the small intestine was examined in 70 patients, with double-balloon endoscopy and ⁄ or capsule endoscopy. The most frequently involved GI-FL site was the duodenal second portion (DSP) (81%), followed by the jejunum (40%); 85% of patients with involvement of the DSP also had jejunal or ileal lesions. The absence of abdominal symptoms and macroscopic appearance of multiple nodules were significantly present in the DSP-positive group. During a median follow up of 40 months, six patients showed disease progression. Patients with involvement of the DSP had better progression-free survival (PFS) than those without such involvement (P = 0.001). A multivariate analysis revealed that male sex, the presence of abdominal symptoms, and negative involvement of the DSP were independently associated with poor PFS. In conclusion, most patients with GI-FL have duodenal lesions associated with multiple jejunal or ileal lesions. Gastrointestinal follicular lymphomas involving the DSP might be a distinct entity showing a favorable clinical course. (Cancer Sci 2011; 102: 1532-1536

Duodenal follicular lymphoma lacks AID but expresses BACH2 and has memory B-cell characteristics

“…Although the duodenal second portion is the most frequent site of FL in the gastrointestinal tract, 3,4 it sometimes occurs primarily in the gastrointestinal tract outside of the duodenum. Using double-balloon and/or capsule endoscopy, FLs of the gastrointestinal tract primarily involve the duodenum and frequently spread to the small intestine, 3 Few FLs occur in the stomach and colon, but the clinicopathological features of FL in such organs remain unclear.…”

Colorectal Manifestation of Follicular Lymphoma

“…Patients with grade 3 follicular lymphoma were excluded from this study because these cases are typically managed as cases of diffuse large B-cell lymphoma (1). A subset of the 12 patients examined herein were also subjects of our previous studies (4,(7)(8)(9)(10)(11)(12)(13)(14)(15)(16).…”

“…It has been widely accepted that follicular lymphoma typically arises in the lymph nodes along with spleen, liver, and bone marrow involvement (1,2). However, several studies have revealed that follicular lymphoma primarily or secondarily affects the gastrointestinal tract as well (3)(4)(5). Involvement of the small intestine, especially the duodenum, is a predominant feature of intestinal presentation in follicular lymphoma.…”

“…In contrast, colorectal involvement is less frequent in cases of follicular lymphoma. For example, Takata and coworkers reported that, among 125 patients with primary intestinal follicular lymphomas, duodenal involvement was found in 111 cases (89%), jejunal involvement in 50 cases (40%) and ileal involvement in 28 cases (22%), whereas involvement of the cecum was seen in only two cases (2%), the colon in one case (1%), and the rectum in two cases (2%) (4).…”

Gastrointestinal Lymphomas: Entities and Mimics

“…It typically presents in the second portion of the duodenum, and less-frequently, in the jejunum 1 and appears endoscopically as one or more small polyps. Although typically asymptomatic, it may present with bowel obstruction.…”