Primary gastrointestinal t-cell lymphoma and indolent lymphoproliferative disorders: Practical diagnostic and treatment approaches

Midori Filiz Nishimura, Yoshito Nishimura, Asami Nishikori, Tadashi Yoshino, Yasuharu Sato

Research output: Contribution to journalReview articlepeer-review

4 Citations (Scopus)


Primary gastrointestinal (GI) T-cell neoplasms are extremely rare heterogeneous disease entities with distinct clinicopathologic features. Given the different prognoses of various disease subtypes, clinicians and pathologists must be aware of the key characteristics of these neoplasms, despite their rarity. The two most common aggressive primary GI T-cell lymphomas are enteropathy-associated T-cell lymphoma and monomorphic epitheliotropic intestinal T-cell lymphoma. In ad-dition, extranodal natural killer (NK)/T-cell lymphoma of the nasal type and anaplastic large cell lymphoma may also occur in the GI tract or involve it secondarily. In the revised 4th World Health Organization classification, indolent T-cell lymphoproliferative disorder of the GI tract has been incor-porated as a provisional entity. In this review, we summarize up-to-date clinicopathological features of these disease entities, including the molecular characteristics of primary GI T-cell lymphomas and indolent lymphoproliferative disorders. We focus on the latest treatment approaches, which have not been summarized in existing reviews. Further, we provide a comprehensive review of available literature to address the following questions: How can pathologists discriminate subtypes with different clinical prognoses? How can primary GI neoplasms be distinguished from secondary involvement? How can these neoplasms be distinguished from non-specific inflammatory changes at an early stage?.

Original languageEnglish
Article number5774
Issue number22
Publication statusPublished - Nov 1 2021


  • EATL
  • Enteropathy-associated T-cell lymphoma
  • Indolent T-cell lymphopro-liferative disorder
  • Monomorphic epitheliotropic intestinal T-cell lymphoma
  • NK-cell enteropathy
  • Primary gastrointestinal T-cell lymphoma

ASJC Scopus subject areas

  • Oncology
  • Cancer Research


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