Search Database ICD-O-3 Code Lists

Name

Intestinal T-cell lymphoma, NOS (ITCL-NOS)

ICD-O-3 Morphology

9717/3: Enteropathy-associated T-cell lymphoma
Effective 2001 and later

Reportable

for cases diagnosed 1978 and later

Primary Site(s)

Module 7
Most common sites of involvement: jejunum, ileum, duodenum, stomach, colon, outside of GI tract (rare).

See abstractor notes

Coding Manual: Hematopoietic Coding Manual (PDF)

Abstractor Notes

Intestinal T-cell lymphoma, NOS (ITCL-NOS) 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-NOS can be confined to a single organ or involve multiple gastrointestinal sites, with the colon and small intestine being the most common. Dissemination to regional lymph nodes and extragastrointestinal sites may be seen.

EATL is most commonly found in the small intestine, frequently the jejunum, but other segments of the small bowel, and the large intestine may be involved. Dissemination to extragastrointestinal sites is most often to mesenteric and abdominal lymph nodes.

MEITL arises most commonly in the small intestine, and less commonly in the colon, duodenum, or stomach; 20–35% of cases may show multifocal gastrointestinal tumours. Tumor may disseminate to mesenteric lymph nodes.

Diagnostic Confirmation

This histology can be determined by positive histology (including peripheral blood) with or without genetics and/or immunophenotyping. Review the Definitive Diagnostic Methods, Immunophenotyping and Genetics Data sections below, and the instructions in the Hematopoietic Manual for further guidance on assigning Diagnostic confirmation.

Module Rule

None

Alternate Names

Enteropathy type intestinal T-cell lymphoma
Enteropathy-associated T-cell lymphoma (EATL)
Enteropathy-associated T-cell lymphoma, type 1
Enteropathy-associated T-cell lymphoma, type 2
Monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL)

Definition

Intestinal T-cell lymphoma NOS (ITCL-NOS) is an aggressive primary gastrointestinal T-cell lymphoma that lacks the clinicopathological features of defined lymphoma entities arising within the gastrointestinal tract. (WHO 5th edition).

Enteropathy-associated T-cell lymphoma (EATL) is an aggressive primary intestinal T-cell lymphoma of intraepithelial lymphocytes (IELs), which exhibits variable cellular pleomorphism and usually occurs in individuals with coeliac disease (CD). (WHO 5th edition)

Monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL) is an aggressive primary intestinal T-cell lymphoma of intraepithelial T lymphocytes, characterized by monomorphic cytomorphology and epitheliotropism, typically lacking association with coeliac disease. (WHO 5th edition)

Definitive Diagnostic Methods

Cytogenetics
Genetic testing
Histologic confirmation
Immunohistochemistry
Immunophenotyping

Genetics Data

8q24(MYC) amplifications (MEITL)
TR gene rearrangements
JAK-1/STAT3 signaling axis
Gains at 9q34.3-qter (MEITL)
Loss at 16q12.1 (MEITL)

Immunophenotyping

CD3+ (expression/positive)
CD4+ (expression/positive)
TCRαβ and a subset are T-cell receptor–silent
TIA1+ (expression/positive)
CD56- (no expression/negative)
CD5- (no expression/negative)
CD7+ (expression/positive)
CD8- (no expression/negative)
CD103+ (expression/positive)

Treatments

Chemotherapy
Hematologic Transplant and/or Endocrine Procedures
Hormone therapy
Radiation therapy
Surgery

Transformations to

There are no known transformations

Transformations from

There are no known transformations

Same Primaries

Corresponding ICD-10 Codes (Cause of Death codes only)

C84.5 Other and unspecified T-cell lymphomas

Corresponding ICD-10-CM Codes (U.S. only)

C86.2 Enteropathy-type (intestinal) T-cell lymphoma (effective October 01, 2015 - September 30, 2024)
C86.20 Enteropathy-type (intestinal) T-cell lymphoma (effective October 01, 2024 - October 01, 2024)
C86.21 Enteropathy-type (intestinal) T-cell lymphoma (effective October 01, 2015)

Signs and Symptoms

Abdominal pain (primarily due to intestinal perforation)
Drenching night sweats
Fatigue
Fever (for no known reason)
Pain in the chest, abdomen, or bones (for no known reason)
Painless swelling in the lymph nodes
Skin rash or itchy skin
Ulcerative jejunitis
Weight loss (for no known reason)

Diagnostic Exams

Blood chemistry studies
Bone marrow aspiration and biopsy
CT (CAT) scan
Cytogenetic analysis
Flow cytometry
Immunohistochemistry
Immunophenotyping
PET (positron emission tomography) scan

Progression and Transformation

Recurrences most frequent in the small intenstine

Epidemiology and Mortality

Survival: poor, death usually results from abdominal complications
Incidence: 3% of peripheral T-cell lymphomas
Age: Median age 61
Sex: M:F ratio 1.04-2.8

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: 717-726

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

PDQ® Adult Treatment Editorial Board. PDQ Peripheral T-Cell Non-Hodgkin Lymphoma Treatment. Bethesda, MD: National Cancer Institute. Updated <03/13/2025>. Available at: https://www.cancer.gov/types/lymphoma/hp/peripheral-t-cell-lymphoma-pdq. Accessed <03/31/2025>. [PMID: 37437079]
Section: Peripheral T-Cell Non-Hodgkin Lymphoma Treatment (PDQ®)–Health Professional Version
Pages: https://www.cancer.gov/types/lymphoma/hp/peripheral-t-cell-lymphoma-pdq#_2289
Glossary