title: Pediatric follicular lymphoma: a report of the first 3 cases from Taiwan and literature review.
abstract: Follicular lymphoma (FL) is a common lymphoma entity in adults but is rare in children. As opposed to adult cases, pediatric FL is characterized by a high-grade histology, low-stage disease, a lower frequency of both bcl-2 protein expression and BCL2 gene rearrangement, and a more favorable prognosis. During the authors' previous study of pediatric Burkitt lymphoma, they identified 3 cases of pediatric FL. Here the authors present the first series of pediatric FL from Taiwan. The patients were 2 boys and 1 girl, aged from 7 to 14. The presentation sites were cervical lymph node in 2 and tonsil in 1. All cases showed large neoplastic nodules comprising sheets of centroblasts, corresponding to grade 3b FL. Two of the 3 tumors weakly expressed bcl-2 protein. Fluorescence in situ hybridization for IGH, BCL2, BCL6, CCND1, and MYC loci showed that the only chromosomal translocation was rearranged IGH in 1 case. Two patients were at stage I, and 1 at stage III. All were treated with combination chemotherapy and achieved long-term complete remission. Literature review including the current cases showed that 45% cases of pediatric FL expressed bcl-2 protein and 9% cases carried BCL2 gene rearrangement, suggesting an alternate molecular pathogenesis of pediatric FL as compared to their adult counterparts.
authors.affiliation: Department of Pathology, Kaohsiung Municipal Ta-Tung Hospital, Kaohsiung Medical University Hospital and Faculty of Medicine, College of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan.
authors.forename: Sheau-Fang
authors.initials: SF
authors.lastname: Yang
authors.forename: Wen-Yu
authors.initials: WY
authors.lastname: Chuang
authors.forename: Seng-Tsung
authors.initials: ST
authors.lastname: Chang
authors.forename: Hongxiang
authors.initials: H
authors.lastname: Liu
authors.forename: Rong-Long
authors.initials: RL
authors.lastname: Chen
authors.forename: Shih-Sung
authors.initials: SS
authors.lastname: Chuang
mesh_terms: D002648:Child; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007143:Immunoglobulin Heavy Chains / Q000235:genetics; D017404:In Situ Hybridization, Fluorescence; D008224:Lymphoma, Follicular / Q000175:diagnosis / Q000188:drug therapy / Q000235:genetics; D008297:Male; D009367:Neoplasm Staging; D012074:Remission Induction; D013624:Taiwan
chemical_list: D007143:Immunoglobulin Heavy Chains