Compendium of Cancer Genome Aberrations

HAEM5:Extranodal NK/T-cell lymphoma: Difference between revisions

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==Definition / Description of Disease==
==Definition / Description of Disease==


Extranodal NK/T-cell lymphoma (ENKTL) is a distinct entity in the [https://tumourclassification.iarc.who.int/chaptercontent/63/258 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<ref name=":5">Chan J. K. C., et al., (2017). Extranodal NK/T-cell lymphoma, nasal type, in World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues, Revised 4th edition. Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Arber DA, Hasserjian RP, Le Beau MM, Orazi A, and Siebert R, Editors. Revised 4th Edition. IARC Press: Lyon, France, p.368-371.</ref>.
Extranodal NK/T-cell lymphoma (ENKTL) is a distinct entity in the [https://tumourclassification.iarc.who.int/chaptercontent/63/258 5th edition World Health Organization (WHO) classification system]. It was 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<ref name=":5">Chan J. K. C., et al., (2017). Extranodal NK/T-cell lymphoma, nasal type, in World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues, Revised 4th edition. Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Arber DA, Hasserjian RP, Le Beau MM, Orazi A, and Siebert R, Editors. Revised 4th Edition. IARC Press: Lyon, France, p.368-371.</ref>.


*ENKTL is a lymphoma of NK or T-cell lineage strongly associated with Epstein-Barr virus<ref name=":6">{{Cite journal|last=Jaffe|first=E. S.|last2=Krenacs|first2=L.|last3=Kumar|first3=S.|last4=Kingma|first4=D. W.|last5=Raffeld|first5=M.|date=1999-01|title=Extranodal peripheral T-cell and NK-cell neoplasms|url=https://pubmed.ncbi.nlm.nih.gov/9894469|journal=American Journal of Clinical Pathology|volume=111|issue=1 Suppl 1|pages=S46–55|issn=0002-9173|pmid=9894469}}</ref>.
*ENKTL is a lymphoma of NK or T-cell lineage strongly associated with Epstein-Barr virus<ref name=":6">{{Cite journal|last=Jaffe|first=E. S.|last2=Krenacs|first2=L.|last3=Kumar|first3=S.|last4=Kingma|first4=D. W.|last5=Raffeld|first5=M.|date=1999-01|title=Extranodal peripheral T-cell and NK-cell neoplasms|url=https://pubmed.ncbi.nlm.nih.gov/9894469|journal=American Journal of Clinical Pathology|volume=111|issue=1 Suppl 1|pages=S46–55|issn=0002-9173|pmid=9894469}}</ref>.
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==Synonyms / Terminology==
==Synonyms / Terminology==


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


==Epidemiology / Prevalence==
==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<ref>{{Cite journal|last=Haverkos|first=Bradley M.|last2=Pan|first2=Zenggang|last3=Gru|first3=Alejandro A.|last4=Freud|first4=Aharon G.|last5=Rabinovitch|first5=Rachel|last6=Xu-Welliver|first6=Meng|last7=Otto|first7=Brad|last8=Barrionuevo|first8=Carlos|last9=Baiocchi|first9=Robert A.|date=2016-12|title=Extranodal NK/T-cell lymphoma, nasal type (ENKTL-NT): An update on epidemiology, clinical presentation, and natural history in North American and European cases|url=https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5199232/|journal=Current hematologic malignancy reports|volume=11|issue=6|pages=514–527|doi=10.1007/s11899-016-0355-9|issn=1558-8211|pmc=5199232|pmid=27778143}}</ref>.
* Most prevalent in East Asia and Latin America.  
* Represents less than 1% of non-Hodgkin lymphomas in the United States
** Highest incidence among Asian Pacific Islanders and Hispanic populations<ref>{{Cite journal|last=Haverkos|first=Bradley M.|last2=Pan|first2=Zenggang|last3=Gru|first3=Alejandro A.|last4=Freud|first4=Aharon G.|last5=Rabinovitch|first5=Rachel|last6=Xu-Welliver|first6=Meng|last7=Otto|first7=Brad|last8=Barrionuevo|first8=Carlos|last9=Baiocchi|first9=Robert A.|date=2016-12|title=Extranodal NK/T-cell lymphoma, nasal type (ENKTL-NT): An update on epidemiology, clinical presentation, and natural history in North American and European cases|url=https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5199232/|journal=Current hematologic malignancy reports|volume=11|issue=6|pages=514–527|doi=10.1007/s11899-016-0355-9|issn=1558-8211|pmc=5199232|pmid=27778143}}</ref>.


==Clinical Features==
==Clinical Features==
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==Sites of Involvement==
==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<ref name=":0" />. Bone marrow involvement is uncommon<ref>{{Cite journal|last=Wong|first=K. F.|last2=Chan|first2=J. K.|last3=Cheung|first3=M. M.|last4=So|first4=J. C.|date=2001-02|title=Bone marrow involvement by nasal NK cell lymphoma at diagnosis is uncommon|url=https://pubmed.ncbi.nlm.nih.gov/11211616|journal=American Journal of Clinical Pathology|volume=115|issue=2|pages=266–270|doi=10.1309/E5PR-6A9R-Q02N-8QVW|issn=0002-9173|pmid=11211616}}</ref>.
* Most are nasal type involving the upper aerodigestive tract
* Extranasal type may involve skin, testis, and gastrointestinal tract<ref name=":0" />.  
* Bone marrow involvement is uncommon<ref>{{Cite journal|last=Wong|first=K. F.|last2=Chan|first2=J. K.|last3=Cheung|first3=M. M.|last4=So|first4=J. C.|date=2001-02|title=Bone marrow involvement by nasal NK cell lymphoma at diagnosis is uncommon|url=https://pubmed.ncbi.nlm.nih.gov/11211616|journal=American Journal of Clinical Pathology|volume=115|issue=2|pages=266–270|doi=10.1309/E5PR-6A9R-Q02N-8QVW|issn=0002-9173|pmid=11211616}}</ref>.


==Morphologic Features==
==Morphologic Features==
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[[File:Extranodal NK T-cell lymphoma, nasal type.vsi(17.8X) snapshot.png|thumb|Extranodal NK T-cell lymphoma, nasal type (HPS). Angiocentric and angiodestructive growth pattern.]]
[[File:Extranodal NK T-cell lymphoma, nasal type.vsi(17.8X) snapshot.png|thumb|Extranodal NK T-cell lymphoma, nasal type (HPS). Angiocentric and angiodestructive growth pattern.]]


*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.
*Diffuse infiltrate composed of admixture of small, medium, or large and anaplastic cells.  
*Cells have irregularly folded nuclei and moderate pale cytoplasm.
*Loss of mucosal glands.
*Loss of mucosal glands.
*Angiocentric and angiodestructive growth pattern.
*Angiocentric and angiodestructive growth pattern with coagulative necrosis.
*Coagulative necrosis and apoptosis.
*Usually see apoptotic cells and mitotic figures
*Mitotic figures.


Pitfalls:
Pitfalls:
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==Genetic Diagnostic Testing Methods==
==Genetic Diagnostic Testing Methods==


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


==Familial Forms==
==Familial Forms==
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