Intestinal T-cell Lymphoma, Not Otherwise Specified (NOS)

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editPREVIOUS EDITION
This page from the 4th edition of Haematolymphoid Tumours is being updated. See 5th edition Table of Contents.

Primary Author(s)*

Derick Okwan MD, PhD (Stanford Medicine, CA)

Sumire Kitahara MD (Cedars-Sinai, CA)

Cancer Category/Type

HAEM4:Mature T- and NK-cell Neoplasms

Cancer Sub-Classification / Subtype

HAEM4:Intestinal T-cell Lymphoma

Definition / Description of Disease

Synonyms / Terminology

  • N/A

Epidemiology / Prevalence

  • N/A

Clinical Features

  • Aggressive disease with typically widespread involvement[1]
  • Not associated with celiac disease[1]
  • Weight loss, anorexia, fatigue, possible intestinal obstruction/perforation[1]
  • May not respond to standard therapy indicated for enteropathy-associated T-cell lymphoma or monomorphic epitheliotropic intestinal T-cell lymphoma[1]

Sites of Involvement

  • Intestinal tract (predominantly colon)

Morphologic Features

  • Heterogeneous, variable-sized lymphocytic infiltrate
  • Shares features compatible with both enteropathy-associated T-cell lymphoma and monomorphic epitheliotropic intestinal T-cell lymphoma

Immunophenotype

  • N/A

Chromosomal Rearrangements (Gene Fusions)

  • No unique disease-specific genetic features

Characteristic Chromosomal Aberrations / Patterns

  • No unique disease-specific chromosomal pattern

Genomic Gain/Loss/LOH

  • N/A

Gene Mutations (SNV/INDEL)

  • N/A

Other Mutations

  • N/A

Epigenomics (Methylation)

  • Not described

Genes and Main Pathways Involved

  • N/A

Diagnostic Testing Methods

  • Must rule out definitive diagnosis of enteropathy-associated T-cell lymphoma and monomorphic epitheliotropic intestinal T-cell lymphoma

Clinical Significance (Diagnosis, Prognosis and Therapeutic Implications)

  • Aggressive course
  • Must rule out all definitive diagnoses, including enteropathy-associated T-cell lymphoma and monomorphic epitheliotropic intestinal T-cell lymphoma and indolent T-cell lymphoproliferative disorder of the gastrointestinal tract

Familial Forms

  • None

Other Information

Links

HAEM4:Intestinal T-cell Lymphoma


References

  1. 1.0 1.1 1.2 1.3 Attygalle, Ayoma D; et al. (2014). "Peripheral T-cell and NK-cell lymphomas and their mimics; taking a step forward - report on the lymphoma workshop of the XVIth meeting of the European Association for Haematopathology and the Society for Hematopathology". Histopathology. 64 (2): 171–199. doi:10.1111/his.12251. ISSN 0309-0167. PMC 6364972. PMID 24128129.

Notes

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