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This neoplasm is not reportable.
Name
ICD-O-3 Morphology
Effective
2021 and later
Reportability
This neoplasm is not reportable
Primary Site(s)
No primary site specified
Primary Site: Gastrointestinal tract (C150-C269)
This hematopoietic disease is not reportable for the U.S.
Canada, refer to your specific province for reportability requirements.
Most common primary sites: stomach and colon
This hematopoietic disease is not reportable for the U.S.
Canada, refer to your specific province for reportability requirements.
Most common primary sites: stomach and colon
Abstractor Notes
Indolent T-cell lymphoma of the gastrointestinal tract (iTCL-GI) is part of the Mature T-cell and NK-cell neoplasms lineage table in the WHO 5th edition of Hematolymphoid Tumors. (See Appendix B in the Hematopoietic Manual, Table B20)
iTCL-GI affects mostly the small intestine or colon; however, any site in the GI tract can be involved, including the oral cavity and the esophagus.
Disease progression with extragastrointestinal spread or transformation to aggressive lymphoma can occur, usually decades after diagnosis.
iNK-LPD involves mostly the stomach, small intestine, and large intestine.
iTCL-GI affects mostly the small intestine or colon; however, any site in the GI tract can be involved, including the oral cavity and the esophagus.
Disease progression with extragastrointestinal spread or transformation to aggressive lymphoma can occur, usually decades after diagnosis.
iNK-LPD involves mostly the stomach, small intestine, and large intestine.
Alternate Names
Lymphomatoid gastropathy
NK-cell enteropathy
Definition
Indolent T-cell lymphoma of the gastrointestinal tract (iTCL-GI) is a clonal T-cell proliferation characterized by the infiltration of the lamina propria by small mature lymphocytes lacking significant epitheliotropism, and typically a protracted clinical course. (WHO 5th edition)
Indolent NK-cell lymphoproliferative disorder (iNK-LPD) of the gastrointestinal tract is an indolent but recurring EBV-negative NK-cell proliferation that predominantly involves the gastrointestinal tract but occasionally may affect other anatomical sites. (WHO 5th edition)
Indolent NK-cell lymphoproliferative disorder (iNK-LPD) of the gastrointestinal tract is an indolent but recurring EBV-negative NK-cell proliferation that predominantly involves the gastrointestinal tract but occasionally may affect other anatomical sites. (WHO 5th edition)
Genetics Data
This data item does not apply
Immunophenotyping
This data item does not apply
Corresponding ICD-10 Codes (Cause of Death codes only)
D47.7 Other specified neoplasms of uncertain or unknown behavior of lymphoid, hematopoietic, and related tissue
Corresponding ICD-10-CM Codes (U.S. only)
D47.Z9 Other specified neoplasms of uncertain behavior of lymphoid, hematopoietic and related tissue (effective October 01, 2015)
Signs and Symptoms
Abdominal pain
Diarrhea
Dyspepsia
Vomiting
Weight loss
Epidemiology and Mortality
Sources
WHO Classification of Tumours Editorial Board. Haematolymphoid tumours. Lyon (France): International Agency for Research on Cancer; 2024. (WHO classification of tumours series, 5th ed.; vol. 11). https://publications.iarc.who.int/637.
Section: Mature T-cell and NK-cell neoplasms
Pages: Part B: 712-714
Section: Mature T-cell and NK-cell neoplasms
Pages: Part B: 712-714
International Classification of Diseases for Oncology, 3rd edition (including revisions). Geneva: World Health Organization, 2001, 2011, 2020.
Section: ICD-O-3.2 (2020) Morphological Codes
Pages: http://www.iacr.com.fr/index.php?option=com_content&view=category&layout=blog&id=100&Itemid=577
Section: ICD-O-3.2 (2020) Morphological Codes
Pages: http://www.iacr.com.fr/index.php?option=com_content&view=category&layout=blog&id=100&Itemid=577
