Extranodal NK-/T-cell lymphoma, nasal type

Name
Extranodal NK-/T-cell lymphoma, nasal type
ICD-O-2 Morphology
9713/3
Effective 1992 - 2000
ICD-O-3 Morphology
9719/3
Effective 2001 and later
Reportable
for cases diagnosed 1992 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.

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Grade
8 - NK (natural killer) cell
Module Rule
None
Alternate Names
Angiocentric immunoproliferative lesion
Angiocentric T-cell lymphoma [OBS]
Extranasal NK/T-cell lymphoma
Extranodal natural killer cell/T-cell lymphoma, nasal type
Lethal granuloma
Lethal midline granuloma
Malignant midline reticulosis [OBS]
Malignant reticulosis, NOS [OBS]
NK-/T-cell lymphoma, nasal and nasal-type
T-/NK-cell lymphoma
Polymorphic reticulosis [OBS]
Definition
Predominantly extranodal, characterized by a broad morphologic spectrum. The infiltrate is often angiocentric, with prominent necrosis and vascular destruction. It is designated NK/T (rather than NK) because while most cases appear to be NK cell neoplasms, (EBV+ CD56+), rare cases show EBV+ CD56- cytotoxic T-cell phenotype.
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 disease 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 disease may have involvement of the lymph nodes, bone marrow and peripheral blood.
Definitive Diagnostic Methods
Genetic testing
Histologic confirmation
Immunophenotyping
Genetics Data
T-cell receptor and immunoglobulin genes are in germline configuration in most cases
T-cell receptor genes show clonal rearrangement in small proportion of cases
Immunophenotyping
CD2+
CD4-
CD5-
CD8-
CD16-
CD25-
CD43-
CD45R0-
CD56+
CD57-
Negative: HLA-DR, FAS (CD95), FAS Ig, Surface CD3, TCR delta/beta F1
Positive: Cytoplasmic CD3 epsilon, Granzyme B, perforin, T1A1
Treatments
Chemotherapy
Hormone
Radiation
Stem cell transplant
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 (effective October 1, 2015 U.S. only)
C86.0 Extranodal NK/T-cell lymphoma, nasal type
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
Bone marrow aspiration and biopsy
CT (CAT) scan
Complete blood count (CBC)
MRI (magnetic resonance imaging)
PET (positron emission tomography) scan
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