Extranodal NK/T-cell lymphoma

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Haematolymphoid Tumours (5th ed.)

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Primary Author(s)*

Teodora Popa, MD, Queen's University

Amanda Xu, MD, Queen's University

Cancer Category / Type

Mature T-cell and NK-cell neoplasms

Cancer Sub-Classification / Subtype

EBV-positive T-cell and NK-cell neoplasms

Definition / Description of Disease

Extranodal NK/T-cell lymphoma (ENKTL) is a distinct entity in the 5th edition World Health Organization (WHO) classification system. It is referred to as "extranodal NK/T-cell lymphoma, nasal type" in the 2016 WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues revised 4th edition[1].

  • ENKTL is a lymphoma of NK or T-cell lineage strongly associated with Epstein-Barr virus[2].
  • It is a destructive angiocentric disease characterized by vascular destruction and necrosis[3].
  • It can be clinically divided into nasal and non-nasal types, the latter most often occurring in the skin and intestinal tract[1][2].
  • The lineage (NK or T-cell) has no clinical significance[4].
  • The differential diagnosis includes sinonasal carcinomas and other lymphomas of the nasal cavity, such as diffuse large B-cell lymphoma[5].

Synonyms / Terminology

Extranodal NK/T-cell lymphoma, nasal type; EBV-positive extranodal NK/T-cell lymphoma; angiocentric lymphoma (not recommended); lethal midline granuloma (historical)

Epidemiology / Prevalence

ENKTL is most prevalent in East Asia and Latin America. It represents less than 1% of non-Hodgkin lymphomas in the United States, with the highest incidence among Asian Pacific Islanders and Hispanic populations[6].

Clinical Features

Common clinical presentations of nasal-type ENKTL include nasal mass, obstruction, and bleeding. Patients with abdominal involvement may present with abdominal pain, gastrointestinal (GI) bleed, or perforation[7]. The presence of B symptoms is associated with higher clinical stage[8].

Signs and Symptoms Nasal mass, nasal obstruction, nasal bleeding

Hoarseness, dysphagia, halitosis, airway obstruction, dysphonia

Abdominal pain, GI bleeding, bowel perforation

B symptoms (fever, weight loss, night sweats)

Laboratory Findings No specific findings

Cytopenias

Sites of Involvement

Most cases of ENKTL are nasal type, meaning they involve the upper aerodigestive tract. Extranasal ENKTL may involve the skin, testis, and gastrointestinal tract[7]. Bone marrow involvement is uncommon[9].

Morphologic Features

  • Diffuse lymphomatous infiltrate composed of small, medium, or large and anaplastic cells (or a mix of small and large cells). The cells have irregularly folded nuclei and moderate pale cytoplasm.
  • Loss of mucosal glands.
  • Angiocentric and angiodestructive growth pattern.
  • Coagulative necrosis and apoptosis.
  • Mitotic figures.

Pitfalls:

  • Mucosal ulceration and superimposed inflammation can mimic an inflammatory process, particularly in less aggressive cases[10].
  • Pseudoepitheliomatous hyperplasia of the overlying mucosal epithelium can mimic squamous cell carcinoma[11][12].


***Amanda, do you know if we have any good cases we could scan and add photos of?

Immunophenotype

The majority of cases are positive for cytoplasmic CD3ε, CD2, granzyme B, and TIA-1. Most ENKTLs are of NK-lineage and express CD56. Cases of T-lineage express T-cell receptor (TCR) and show clonal TCR gene rearrangements. All cases are EBV positive by in situ hybridization for Epstein-Barr virus-encoded small RNA (EBER). Other markers that may be expressed include HLA-DR, CD25, pSTAT3, CXCL13, IRF4/MUM1, CD16, Fas, FasL, MATK, and CD30[13][14][15][16][17][18][19].

Finding Marker
Positive (universal) CD2, CD56, cytoplasmic CD3ε, cytotoxic markers (TIA-1, granzyme B, perforin)

EBER / EBV

Positive (subset) TCR αβ/γδ, HLA-DR, CD25, pSTAT3, CXCL13, IRF4/MUM1, CD16, Fas, FasL, MATK, CD30
Negative (universal) CD4, CD8
Negative (subset) Surface CD3 (subset of T-cell lineage)[7]

Chromosomal Rearrangements (Gene Fusions)

No specific chromosomal translocation has been identified in ENKTL.

Chromosomal Rearrangement Genes in Fusion (5’ or 3’ Segments) Pathogenic Derivative Prevalence Diagnostic Significance (Yes, No or Unknown) Prognostic Significance (Yes, No or Unknown) Therapeutic Significance (Yes, No or Unknown) Notes
N/A N/A N/A N/A N/A N/A N/A N/A

Individual Region Genomic Gain / Loss / LOH

ENKTL shows recurring deletion at 6q21-25[20][21].

Other less common chromosomal alterations include gain of 1p, 2q, 6p, 10q, 11q, 12q, 13q, 17q, 19p, 20q, and Xp; and loss of 1p36, 2p16, 4q12, 4q31-32, 5p14, 5q34-35, 6q13-14, 6q16-27, 11q22-23, 12q, 13q12-14, 13q14-34, 17p13, and entire chromosome X[22][23][24][25][26].

Chr # Gain / Loss / Amp / LOH Minimal Region Genomic Coordinates [Genome Build] Minimal Region Cytoband Diagnostic Significance (Yes, No or Unknown) Prognostic Significance (Yes, No or Unknown) Therapeutic Significance (Yes, No or Unknown) Notes
6 Loss 6q21-25 Unknown Unknown Unknown This locus harbours multiple candidate tumour suppressor genes including ATG5, AIM1, PRDM1, PTPRK, HACE1, and FOXO3[27][28][29].

Characteristic Chromosomal Patterns

Chromosomal Pattern Diagnostic Significance (Yes, No or Unknown) Prognostic Significance (Yes, No or Unknown) Therapeutic Significance (Yes, No or Unknown) Notes
Isochromosome 6p[30] Unknown Unknown Unknown N/A
Isochromosome 7q[31] Unknown Unknown Unknown N/A

Gene Mutations (SNV / INDEL)

Recurrent mutations have been identified in several genes, listed below. Currently, molecular testing for these alterations is not routinely performed.

Gene; Genetic Alteration Presumed Mechanism (Tumor Suppressor Gene [TSG] / Oncogene / Other) Prevalence (COSMIC / TCGA / Other) Concomitant Mutations Mutually Exclusive Mutations Diagnostic Significance (Yes, No or Unknown) Prognostic Significance (Yes, No or Unknown) Therapeutic Significance (Yes, No or Unknown) Notes
JAK3[32][33] Oncogene 35.4%[32] Pan-JAK and selective JAK3 inhibitors have been suggested as potential therapeutic options[32][34].
STAT3[35][36][37] 26%[37] STAT3 inhibitor may have potential therapeutic benefit in patients with STAT3 activating mutation[38].
STAT5B[35][36]
PTPRK[29] Other (acts on JAK/STAT pathway; underexpression leads to STAT3 activation[29])
KIT[29] Oncogene
CTNNB1[29] Oncogene
TP53[35] Tumor suppressor gene 24-62%[39][40] Yes, associated with advanced stage disease[39].
MGA[35] Tumor suppressor gene
PRDM1[41][28][42] Tumor suppressor gene
ATG5[41] Tumor suppressor gene
AIM1[41] Tumor suppressor gene
FOXO3[41][28] Tumor suppressor gene
HACE1[41][43] Tumor suppressor gene
RAS[40] Oncogene
MYC Oncogene

Note: A more extensive list of mutations can be found in cBioportal (https://www.cbioportal.org/), COSMIC (https://cancer.sanger.ac.uk/cosmic), ICGC (https://dcc.icgc.org/) and/or other databases. When applicable, gene-specific pages within the CCGA site directly link to pertinent external content.

Epigenomic Alterations

A 2015 study by Lu, et al uncovered recurrent mutations in the RNA helicase gene DDX3X and other epigenetic modifiers including KMT2D (MLL2), ARID1A, EP300, and ASXL3[35].

Similarly, a 2015 study by Lee, et al reported that histone modification-related genes, including BCOR and KMT2D (MLL2), accounted for 38.2% of 34 ENKTL samples by next-generation sequencing[37].

Genes and Main Pathways Involved

Gene; Genetic Alteration Pathway Pathophysiologic Outcome
JAK3, STAT3, and STAT5B; Activating mutations JAK/STAT pathway Increased cell growth and proliferation

Genetic Diagnostic Testing Methods

Not routinely performed. Select cases may require TCR gene rearrangement studies.

Familial Forms

N/A

Additional Information

N/A

Links

5th edition World Health Organization (WHO) classification system

References

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Notes

*Primary authors will typically be those that initially create and complete the content of a page.  If a subsequent user modifies the content and feels the effort put forth is of high enough significance to warrant listing in the authorship section, please contact the CCGA coordinators (contact information provided on the homepage).  Additional global feedback or concerns are also welcome. *Citation of this Page: “Extranodal NK/T-cell lymphoma”. Compendium of Cancer Genome Aberrations (CCGA), Cancer Genomics Consortium (CGC), updated 08/19/2024, https://ccga.io/index.php/HAEM5:Extranodal_NK/T-cell_lymphoma.