Primary Gastrointestinal Follicular Lymphoma: A Clinicopathologic Study of 13 Cases From Taiwan

Abstract

Follicular lymphoma (FL) is a common nodal lymphoma. Primary gastrointestinal follicular lymphoma (GIFL) is rare and most commonly occurs in the elder women with indolent clinical course, mainly in the duodenum and terminal ileum. We conducted this study to characterize GIFL in Taiwan. A total of 13 cases were retrospectively collected in 4 hospitals in southern and eastern Taiwan up to 2006. Pathology review and immunohistochemistry were performed and the clinical data were obtained from chart review. There were 5 men and 8 women with a median age of 58 years (range, 31 to 74; mean, 55). The most frequent site was ileum (7/13, 54%) with 4 tumors in the terminal ileum and the most common symptom was abdominal pain (8/13, 62%). Multiple lymphomatous polyposis was present in 6 patients (46%). Five (45%) of 11 tumors with surgical resection showed transmural involvement. Most of the cases revealed a predominantly follicular pattern (7/13, 54%) and low-grade disease (grades 1 and 2; 10/13, 77%). The tumors expressed CD10 (62%), Bcl-2 (77%), Bcl-6 (77%), and MUM1 (38%). Five patients (38%) were at stage IE and 8 (62%) at stage IIE. Treatment included surgery with or without chemotherapy. All patients achieved complete remission and relapse occurred in 3 patients. At a median follow-up time of 51 months (range, 3 to 180; mean, 63), 11 patients (85%) were disease free and no patient died of lymphoma. We characterized the clinicopathologic features of the first series of GIFL in Taiwan. These tumors frequently involved ileum with a favorable prognosis.