Primary Gastrointestinal T-Cell Lymphoma and Indolent Lymphoproliferative Disorders: Practical Diagnostic and Treatment Approaches

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...

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Autores principales: Midori Filiz Nishimura, Yoshito Nishimura, Asami Nishikori, Tadashi Yoshino, Yasuharu Sato
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Lenguaje:EN
Publicado: MDPI AG 2021
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spelling oai:doaj.org-article:31bf6c2ba5f6423a849da0e6fb3502612021-11-25T17:03:49ZPrimary Gastrointestinal T-Cell Lymphoma and Indolent Lymphoproliferative Disorders: Practical Diagnostic and Treatment Approaches10.3390/cancers132257742072-6694https://doaj.org/article/31bf6c2ba5f6423a849da0e6fb3502612021-11-01T00:00:00Zhttps://www.mdpi.com/2072-6694/13/22/5774https://doaj.org/toc/2072-6694Primary 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 addition, 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 incorporated 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?Midori Filiz NishimuraYoshito NishimuraAsami NishikoriTadashi YoshinoYasuharu SatoMDPI AGarticleprimary gastrointestinal T-cell lymphomaenteropathy-associated T-cell lymphomaEATLmonomorphic epitheliotropic intestinal T-cell lymphomaMEITLindolent T-cell lymphoproliferative disorderNeoplasms. Tumors. Oncology. Including cancer and carcinogensRC254-282ENCancers, Vol 13, Iss 5774, p 5774 (2021)
institution DOAJ
collection DOAJ
language EN
topic primary gastrointestinal T-cell lymphoma
enteropathy-associated T-cell lymphoma
EATL
monomorphic epitheliotropic intestinal T-cell lymphoma
MEITL
indolent T-cell lymphoproliferative disorder
Neoplasms. Tumors. Oncology. Including cancer and carcinogens
RC254-282
spellingShingle primary gastrointestinal T-cell lymphoma
enteropathy-associated T-cell lymphoma
EATL
monomorphic epitheliotropic intestinal T-cell lymphoma
MEITL
indolent T-cell lymphoproliferative disorder
Neoplasms. Tumors. Oncology. Including cancer and carcinogens
RC254-282
Midori Filiz Nishimura
Yoshito Nishimura
Asami Nishikori
Tadashi Yoshino
Yasuharu Sato
Primary Gastrointestinal T-Cell Lymphoma and Indolent Lymphoproliferative Disorders: Practical Diagnostic and Treatment Approaches
description 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 addition, 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 incorporated 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?
format article
author Midori Filiz Nishimura
Yoshito Nishimura
Asami Nishikori
Tadashi Yoshino
Yasuharu Sato
author_facet Midori Filiz Nishimura
Yoshito Nishimura
Asami Nishikori
Tadashi Yoshino
Yasuharu Sato
author_sort Midori Filiz Nishimura
title Primary Gastrointestinal T-Cell Lymphoma and Indolent Lymphoproliferative Disorders: Practical Diagnostic and Treatment Approaches
title_short Primary Gastrointestinal T-Cell Lymphoma and Indolent Lymphoproliferative Disorders: Practical Diagnostic and Treatment Approaches
title_full Primary Gastrointestinal T-Cell Lymphoma and Indolent Lymphoproliferative Disorders: Practical Diagnostic and Treatment Approaches
title_fullStr Primary Gastrointestinal T-Cell Lymphoma and Indolent Lymphoproliferative Disorders: Practical Diagnostic and Treatment Approaches
title_full_unstemmed Primary Gastrointestinal T-Cell Lymphoma and Indolent Lymphoproliferative Disorders: Practical Diagnostic and Treatment Approaches
title_sort primary gastrointestinal t-cell lymphoma and indolent lymphoproliferative disorders: practical diagnostic and treatment approaches
publisher MDPI AG
publishDate 2021
url https://doaj.org/article/31bf6c2ba5f6423a849da0e6fb350261
work_keys_str_mv AT midorifiliznishimura primarygastrointestinaltcelllymphomaandindolentlymphoproliferativedisorderspracticaldiagnosticandtreatmentapproaches
AT yoshitonishimura primarygastrointestinaltcelllymphomaandindolentlymphoproliferativedisorderspracticaldiagnosticandtreatmentapproaches
AT asaminishikori primarygastrointestinaltcelllymphomaandindolentlymphoproliferativedisorderspracticaldiagnosticandtreatmentapproaches
AT tadashiyoshino primarygastrointestinaltcelllymphomaandindolentlymphoproliferativedisorderspracticaldiagnosticandtreatmentapproaches
AT yasuharusato primarygastrointestinaltcelllymphomaandindolentlymphoproliferativedisorderspracticaldiagnosticandtreatmentapproaches
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