Intestinal T-cell Lymphoma, Not Otherwise Specified (NOS)
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editPREVIOUS EDITIONThis 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
- Category that is assigned T-cell lymphoma entities that do not meet diagnostic criteria for HAEM5:Enteropathy-associated T-cell lymphoma or HAEM5:Monomorphic epitheliotropic intestinal T-cell lymphoma
- Typically assigned in the setting of inadequate biopsy, equivocal assessment of mucosal surface, incomplete immunophenotype, or incomplete clinical history where celiac disease is uninvolved
- Not a distinct clinical entity
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
- ↑ 1.0 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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