Search Database ICD-O-3 Code Lists

Name

Extranodal NK-/T-cell lymphoma (ENKTL)

ICD-O-3 Morphology

9719/3: Extranodal NK/T cell lymphoma, nasal type
Effective 2001 and later

Reportable

for cases diagnosed 1978 and later

Primary Site(s)

Module 7
Most common sites of involvement: upper aerodigestive tract (nasal cavity, nasopharynx, paranasal sinuses, palate) with the nasal cavity (C300) being the prototypic site of involvement.

See abstractor notes.

Coding Manual: Hematopoietic Coding Manual (PDF)

Abstractor Notes

Extranodal NK-/T-cell lymphoma (ENKTL) 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)

ENKTLs are subdivided according to the site of primary tumor (not presentation)

Nasal subtype sites include the nasal cavity, nasopharynx, oropharynx, Waldeyer ring, epiglottis, and/or aryepiglottic fold. Lesions in the nasal cavity can invade externally into the face or erode the nasal floor, destroying and perforating the hard palate, resulting in the typical clinical appearance of so-called “lethal midline granuloma”. Regional lymph nodes may be involved. In late stages, systemic spread can occur, with a predilection for the skin, gastrointestinal tract, testis, liver, spleen, and bone marrow.

Non-nasal subtype are those commonly involved in the advanced stages of nasal ENKTL, namely the skin, gastrointestinal tract, testis, and miscellaneous sites.

Strong correlation between ENKTL's and EBV infection.

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

Angiocentric T-cell lymphoma [OBS]
EBV-positive extranodal NK/T-cell lymphoma
Extranodal NK-/T-cell lymphoma, nasal type
Lethal midline granuloma
Malignant midline reticulosis [OBS]
Malignant reticulosis, NOS [OBS]
Nasal extranodal NK-T-cell lymphoma (Nasal ENKTL)
NK-/T-cell lymphoma, nasal and nasal-type
Non-nasal (extranasal) NK/T-cell lymphoma
Polymorphic reticulosis [OBS]

Definition

Extranodal NK/T-cell lymphoma (ENKTL) is an extranodal lymphoma of NK- or T-cell lineage characterized by vascular damage and destruction, prominent necrosis, a cytotoxic phenotype, and an association with EBV. (WHO 5th edition)

Definitive Diagnostic Methods

Genetic testing
Histologic confirmation
Immunophenotyping

Genetics Data

Epigenetic regulators (BCOR, KMT2D, ARID1A, EP300)
Recurrent mutations JAK/STAT pathway genes (STAT3, JAK3, STAT5B)
Tumor suppressor genes (TP53, MGA), and RNA helicase (DDX3X)
6q21-25 chromosomal aberration (harbors suppressor genes PRDM1, PTPRK, HACE1, and FOXO3)

Immunophenotyping

CD2+ (expression/positive)
sCD3- (no expression/negative)
cCD3ε+ (expression/positive))
CD5- (no expression/negative)
CD7+ (expression/positive)
CD8 - (no expression/negative)
CD16- (no expression/negative)
CD25- (no expression/negative)
CD30+ (expression/positive)
CD43+ (expression/positive)
CD45R0+ (expression/positive)
CD56+ (expression/positive)
CD57- (no expression/negative)
FASL (CD25)+ (expression/positive)
Positive cytotoxic molecules positive (TIA1, Granzyme B, Perforin)

Treatments

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

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)

C86.0 Extranodal NK/T-cell lymphoma, nasal type (effective October 01, 2015)
C84.9 Mature T/NK-cell lymphomas, unspecified (effective October 01, 2015)

Signs and Symptoms

Drenching night sweats
Fatigue
Fever (for no known reason)
Midfacial destructive lesions
Nasal obstruction due to mass
Pain in the chest, abdomen, or bones (for no known reason)
Painless swelling in the lymph nodes
Ulcerated nodular skin lesions
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
Lymph node biopsy
PET (positron emission tomography) scan

Progression and Transformation

None

Epidemiology and Mortality

Incidence: low (predominantly occurs in Asia)
Age: more common in adults
Sex: male predominance
Survival: 30-40%. Poorer survival time when occurring outside of the nasal cavity

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- and NK-cell neoplasms
Pages: Part B: 765-769

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