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

Ren Ching Wang, Bo Jung Chen, Chang Tsu Yuan, Chung Han Ho, Wen Yu Chuang, Shang Wen Chen, Julia Hueimei Chang, Wei Hsiang Yu, Shih Sung Chuang

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Abstract

Primary 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.

Original languageEnglish
Article number154184
JournalPathology Research and Practice
Volume240
DOIs
Publication statusPublished - Dec 2022

Keywords

  • Enteropathy-associated T-cell lymphoma (EATL)
  • Intestinal perforation
  • Monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL)
  • Primary intestinal peripheral T-cell lymphoma, not otherwise specified (ITCL-NOS)
  • Primary intestinal T-cell lymphoma (PITL)

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Cell Biology

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