Name

Extranodal NK-/T-cell lymphoma, nasal type

ICD-O-2 Morphology

9713/3: Angiocentric T-cell lymphoma

ICD-O-3 Morphology

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

Reportable

for cases diagnosed 2001 and later

Primary Site(s)

See 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.

Grade

Not Applicable

Module Rule

None

Alternate Names

Angiocentric immunoproliferative lesion
Angiocentric T-cell lymphoma [OBS]
Extranasal NK/T-cell lymphoma
Lethal granuloma
Lethal midline granuloma [OBS]
Malignant midline reticulosis [OBS]
Malignant reticulosis, NOS [OBS]
NK-/T-cell lymphoma, nasal and nasal-type
T-/NK-cell lymphoma
Polymorphic reticulosis [OBS]

Definition

Extranodal NK-/T-cell lymphoma, nasal type, is a predominantly extranodal lymphoma of NK-cell or T-cell lineage, characterized by vascular damage and destruction, prominent necrosis, cytotoxic phenotype, and association with EBV. It is designated as NK/T-cell lymphoma because although most cases appear to be genuine NK-cell neoplasms, some cases are of cytotoxic T-cell lineage.

Abstractor Notes

Extranodal NK/T cell lymphoma almost always shows an extranodal presentation. Some cases may be accompanied by secondary LN involvement although rare instances of primary LN involvement in the absence of extranodal involvement has been reported.

Extension to adjacent tissues such as the nasopharynx, paranasal sinuses, orbit, oral cavity, palate, and oropharynx are possible. At presentation, the disease is often localized to the upper aerodigestive tract.

Disseminated involvement may have involvement of the lymph nodes, bone marrow and peripheral blood.

Definitive Diagnostic Methods

Genetic testing
Histologic confirmation
Immunophenotyping

Genetics Data

IG genes are clonally rearranged
TR genes are clonally rearranged

Immunophenotyping

CD2 positive
CD3 negative or epsilon positive
CD4 negative
CD5 negative
CD7 expression
CD8 negative
CD16 negative
CD25-
CD30 positive
CD43 positive
CD45R0 positive
CD56 positive
CD57 negative
FASL (CD25) expressed
Granzyme B positive
Perforin negative
TIA1 positive

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

Corresponding ICD-9 Codes

202.8 Other lymphoma

Corresponding ICD-10 Codes

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)

Signs and Symptoms

Drenching night sweats
Fever (for no known reason)
Midfacial destructive lesions
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)

Progression and Transformation

None

Epidemiology and Mortality

Age: more common in adults
Race: more prevalent in Asians and Native Americans
Sex: male predominance
Survival: 30-40%. Poorer survival time when occurring outside of the nasal cavity

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: 368-371

International Classification of Diseases for Oncology, Third Edition, First Revision. Geneva: World Health Organization, 2013.
Section: ICD-O-3.1 (2011) Morphological Codes
Pages: http://codes.iarc.fr/codegroup/2

National Cancer Institute
Section: General Information About Adult Non-Hodgkin Lymphoma (NHL)
Pages: https://www.cancer.gov/types/lymphoma/hp/adult-nhl-treatment-pdq
Glossary