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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
This neoplasm is reportable for primary sites C700-C729, C751-C753 only.
Abstractor Notes
Most patients present with disease affecting the small bowel or colon. All sites in the gastrointestinal tract can be involved though, including the oral cavity and esophagus. The bone marrow and peripheral blood are usually not involved.
The clinical course is chronic, but progression to disseminated disease has been reported, but is infrequent.
The clinical course is chronic, but progression to disseminated disease has been reported, but is infrequent.
Definition
Indolent T-cell lymphoproliferative disorder of the gastrointestinal tract is a clonal T-cell lymphoproliferative disorder that can involve the mucosa in all sites of the gastrointestinal tract, but is most common in the small intestine and colon. The lymphoid cells infiltrate the lamina propria but usually do not show invasion of the epithelium. The clinical course is indolent, but most patients do not respond to conventional chemotherapy. A subset of cases progress to a higher-grade T-cell lymphoma with spread beyond the gastrointestinal tract.
Genetics Data
This data item does not apply
Immunophenotyping
This data item does not apply
Corresponding ICD-9 Codes
238.79 Other lymphatic and hematopoietic tissues
Corresponding ICD-10 Codes
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
Sources
Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J (Eds):
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition)
IARC: Lyon 2017
Section: Mature T- and NK-cell neoplasms
Pages: 379-380
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition)
IARC: Lyon 2017
Section: Mature T- and NK-cell neoplasms
Pages: 379-380
International Classification of Diseases for Oncology, Third Edition, Second Revision. Geneva: World Health Organization, 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
