Research ArticlesPrimary Intestinal Diffuse Large B-cell Lymphoma in Taiwan Showed a Relative Higher Rate of Perforation and EBV AssociationLu, Yi-Hsuan MD*; Chang, Sheng-Tsung MD†,‡; Yang, Sheau-Fang MD§; Weng, Shih-Feng PhD∥; Huang, Wan-Ting MD, PhD¶,#; Hsieh, Pin-Pen MD**; Hsu, Jeng-Dong DMD, MD††; Tsou, Mei-Hua MD‡‡; Chuang, Shih-Sung MD†,§§Author Information Departments of *Radiology, Chi Mei Medical Center, Liouying †Pathology ∥Medical Research, Chi-Mei Medical Centre, Tainan, Taiwan ‡Department of Nursing, National Tainan Institute of Nursing, Tainan, Taiwan §Department of Pathology, Kaohsiung Municipal Ta-Tung Hospital and School of Medicine, Kaohsiung Medical University ¶Department of Pathology, Kaohsiung Chang Gung Memorial Hospital-Kaohsiung Medical Centre #Chang Gung University College of Medicine **Department of Pathology and Laboratory Medicine, Kaohsiung Veterans General Hospital and Centre for General Education, Yuh-Ing Junior College of Health Care and Management, Kaohsiung ††Department of Pathology, Chung Shan Medical University Hospital and School of Medicine, Chung Shan Medical University, Taichung ‡‡Department of Pathology and Laboratory Medicine, Koo Foundation Sun Yat-Sen Cancer Centre §§Department of Pathology, Taipei Medical University and National Taiwan University, Taipei, Taiwan Presented as a poster at the 17th Meeting of the European Association for Hematopathology (EAHP), October 17 to 22, 2014, Istanbul, Turkey. Supported by the research grant CLFHR10201 (to Y.-H.L) from Chi Mei Medical Center, Liouying, Tainan, NSC 101-2314-B-384 -006 and MOST 103-2314-B-384-003 (to S.-S.C.) from Ministry of Science and Technology, Taipei, Taiwan. S.-S.C. designed the study. S.-T.C., S.-F.Y., W.-T.H., P.-P.H., J.-D.H., and M.-H.T. contributed the cases and analyzed the data. S.-F.W. analyzed the data. Y.-H.L., S.-T.C., and S.-S.C. wrote the manuscript. S.-S.C. revised and finalized the manuscript. Y.-H.L. and S.-T.C. contributed equally and both should be considered first authors. The authors declare no conflicts of interest. Reprints: Shih-Sung Chuang, MD, Department of Pathology, Chi-Mei Medical Centre, 901 Chung-Hwa Road, Yong-Kang District, Tainan 71004, Taiwan (e-mail: [email protected]). Applied Immunohistochemistry & Molecular Morphology: September 2016 - Volume 24 - Issue 8 - p 541-549 doi: 10.1097/PAI.0000000000000226 Buy SDC Metrics Abstract We retrospectively investigated 59 surgically resected primary intestinal diffuse large B-cell lymphomas (PI-DLBCL) including 31 males and 28 females with a median age of 66. Eleven (19%) tumors were perforated at presentation; 8 (14%) were multicentric. Ileum (n=24; 43%) and ileocecum (n=17; 30%) were most frequently involved. Twenty-one (36%) patients did not receive chemotherapy or radiotherapy including 6 with perforation and died in 0.2 to 7 months. The 1-, 2-, and 5-year overall survival rates were 68.4%, 56.5%, and 50.0%, respectively. Seven (12%) of 59 cases were positive for Epstein-Barr virus (EBV) by in situ hybridization. IGH, BCL2, BCL6, and MYC foci were rearranged in 22%, 3%, 17%, and 7% cases, respectively, with 14% exhibiting gain/amplification at the MYC locus. Perforation (P=0.009), high ECOG PS (≥2) (P=0.018), and no adjuvant chemotherapy (P<0.001) were poor prognostic factors but not immunophenotype including co-expression of bcl-2 and myc, EBV status, or chromosomal aberrations. Perforation and chemotherapy remained significant by multivariate analysis. PI-DLBCL in Taiwan carried a relatively higher rate of perforation, lower frequency of germinal center B-cell phenotype, and higher EBV association as compared with studies from other geographic areas. Furthermore, perforation was a poor prognostic factor. Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved.