The spectrum of intestinal mature T- and NK-cell neoplasms in a tertiary center in Taiwan with a high frequency of perforation

Wang, Ren ChingRen ChingWangChen, Bo-JungBo-JungChenCHANG-TSU YUANHo, Chung-HanChung-HanHoChuang, Wen-YuWen-YuChuangChen, Shang-WenShang-WenChenChang, Julia HueimeiJulia HueimeiChangYu, Wei-HsiangWei-HsiangYuChuang, Shih-SungShih-SungChuang2023-02-142023-02-142022-120344-0338https://scholars.lib.ntu.edu.tw/handle/123456789/627950Primary intestinal T-cell lymphomas (PITLs) comprise enteropathy-associated T-cell lymphoma (EATL), monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL), extranodal NK/T-cell lymphoma (ENKTL), anaplastic large cell lymphoma (ALCL), and intestinal T cell lymphoma, NOS (ITCL-NOS). MEITL is composed of monomorphic medium cells expressing CD8 and CD56, with a cytotoxic phenotype. We retrospectively analyzed 77 cases of intestinal T-cell lymphomas, 71 primary and six secondary, at a tertiary center in Taiwan from 2001 to 2021. Perforation occurred in 57 (74%) patients, including 56 (73%) at presentation and one after chemotherapy. The primary cases included MEITL (68%), ENKTL (14%), ITCL-NOS (13%), ALCL (4%), and EATL (1%). The perforation rate was 90%, 70%, and 22% in MEITL, ENKTL, and ITCL-NOS cases, respectively (p < 0.0001, Fisher's exact test). Most (75%; n = 36) MEITL cases were typical; while seven (15%) had atypical morphology and five (10%) exhibited atypical immunophenotype. The tumor cells of ITCL-NOS were pleomorphic, with various expression of CD8 or CD56. All METIL, ITCL-NOS and ALCL cases were negative for EBER; while all ENKTL cases, either primary or secondary, were positive for cytotoxic granules and EBER. The prognosis of PITL was poor, with a medium survival of 7.0, 3.3, and 3.7 months among patients with MEITL, ENKTL, and ITCL-NOS, respectively. Of the six secondary cases, the primary tumors orginated from nasal ENKTL (n = 5) and cutaneous PTCL-NOS (n = 1). We showed a wide spectrum of intestinal T-cell lymphomas in Taiwan, with MEITL as the most common PITL, a high rate of perforation, and a wider morphological and immunophenotypic spectrum.enEnteropathy-associated T-cell lymphoma (EATL); Intestinal perforation; Monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL); Primary intestinal T-cell lymphoma (PITL); Primary intestinal peripheral T-cell lymphoma, not otherwise specified (ITCL-NOS)[SDGs]SDG1[SDGs]SDG3The spectrum of intestinal mature T- and NK-cell neoplasms in a tertiary center in Taiwan with a high frequency of perforationjournal article10.1016/j.prp.2022.154184363278202-s2.0-85140452240WOS:000881797200002https://api.elsevier.com/content/abstract/scopus_id/85140452240