HAEM5:Extranodal NK/T-cell lymphoma: Difference between revisions
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==WHO Essential and Desirable Genetic Diagnostic Criteria== | ==WHO Essential and Desirable Genetic Diagnostic Criteria== | ||
<span style="color:#0070C0">(''Instructions: The table will have the diagnostic criteria from the WHO book <u>autocompleted</u>; remove any <u>non</u>-genetics related criteria. If applicable, add text about other classification'' ''systems that define this entity and specify how the genetics-related criteria differ.'')</span> | <span style="color:#0070C0">(''Instructions: The table will have the diagnostic criteria from the WHO book <u>autocompleted</u>; remove any <u>non</u>-genetics related criteria. If applicable, add text about other classification'' ''systems that define this entity and specify how the genetics-related criteria differ.'')</span> | ||
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==Additional Information== | ==Additional Information== | ||
[[File:Extranodal NK T-cell lymphoma, nasal type.png|thumb|Extranodal NK T-cell lymphoma, nasal type (HPS). Angiocentric and angiodestructive growth pattern.]]This disease is <u>defined/characterized</u> as detailed below: | |||
*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>. The lineage (NK or T-cell) has no clinical significance<ref name=":16">{{Cite journal|last=Wang|first=Hua|last2=Fu|first2=Bi-Bo|last3=Gale|first3=Robert Peter|last4=Liang|first4=Yang|date=2021-09|title=NK-/T-cell lymphomas|url=https://pubmed.ncbi.nlm.nih.gov/34117356|journal=Leukemia|volume=35|issue=9|pages=2460–2468|doi=10.1038/s41375-021-01313-2|issn=1476-5551|pmc=8410593|pmid=34117356}}</ref>. | |||
*Divided into nasal and non-nasal types, the latter most often occurring in the skin and intestinal tract<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><ref name=":6" />. | |||
*It is a destructive angiocentric disease characterized by vascular destruction and necrosis<ref name=":17">{{Cite journal|last=Aviles|first=A.|last2=Rodriguez|first2=L.|last3=Guzman|first3=R.|last4=Talavera|first4=A.|last5=Garcia|first5=E. L.|last6=Diaz-Maqueo|first6=J. C.|date=1992|title=Angiocentric T-cell lymphoma of the nose, paranasal sinuses and hard palate|url=https://pubmed.ncbi.nlm.nih.gov/1398510|journal=Hematological Oncology|volume=10|issue=3-4|pages=141–147|doi=10.1002/hon.2900100303|issn=0278-0232|pmid=1398510}}</ref>. | |||
*Differential diagnosis: sinonasal carcinomas and other lymphomas of the nasal cavity, such as diffuse large B-cell lymphoma<ref name=":18">{{Cite journal|last=Steele|first=Toby O.|last2=Buniel|first2=Maria C.|last3=Mace|first3=Jess C.|last4=El Rassi|first4=Edward|last5=Smith|first5=Timothy L.|date=2016-09|title=Lymphoma of the nasal cavity and paranasal sinuses: A case series|url=https://pubmed.ncbi.nlm.nih.gov/27657899|journal=American Journal of Rhinology & Allergy|volume=30|issue=5|pages=335–339|doi=10.2500/ajra.2016.30.4347|issn=1945-8932|pmid=27657899}}</ref>. | |||
*Differential diagnosis: sinonasal carcinomas and other lymphomas of the nasal cavity, such as diffuse large B-cell lymphoma<ref name=":18" />. | |||
The <u>epidemiology/prevalence</u> of this disease is detailed below: | The <u>epidemiology/prevalence</u> of this disease is detailed below: | ||
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*Most prevalent in East Asia and Latin America. | *Most prevalent in East Asia and Latin America. | ||
*Represents less than 1% of non-Hodgkin lymphomas in the United States | *Represents less than 1% of non-Hodgkin lymphomas in the United States | ||
**Highest incidence among Asian Pacific Islanders and Hispanic populations<ref name=":19" />. | **Highest incidence among Asian Pacific Islanders and Hispanic populations<ref name=":19">{{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>. | ||
The <u>clinical features</u> of this disease are detailed below: | The <u>clinical features</u> of this disease are detailed below: [[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.]] | ||
Signs and symptoms - Nasal mass, nasal obstruction, nasal bleeding; Hoarseness, dysphagia, halitosis, airway obstruction, dysphonia; Abdominal pain, GI bleeding, bowel perforation<ref name=":0" />; B symptoms (fever, weight loss, night sweats) associated with higher clinical stage<ref name=":20" /> | Signs and symptoms - Nasal mass, nasal obstruction, nasal bleeding; Hoarseness, dysphagia, halitosis, airway obstruction, dysphonia; Abdominal pain, GI bleeding, bowel perforation<ref name=":0">Thida AM, Gohari P. Extranodal NK-Cell Lymphoma. [Updated 2023 Jul 17]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2024 Jan-. Available from: <nowiki>https://www.ncbi.nlm.nih.gov/books/NBK559207/</nowiki></ref>; B symptoms (fever, weight loss, night sweats) associated with higher clinical stage<ref name=":20">{{Cite journal|last=Takahara|first=Miki|last2=Kumai|first2=Takumi|last3=Kishibe|first3=Kan|last4=Nagato|first4=Toshihiro|last5=Harabuchi|first5=Yasuaki|date=2021-06-25|title=Extranodal NK/T-Cell Lymphoma, Nasal Type: Genetic, Biologic, and Clinical Aspects with a Central Focus on Epstein-Barr Virus Relation|url=https://pubmed.ncbi.nlm.nih.gov/34202088|journal=Microorganisms|volume=9|issue=7|pages=1381|doi=10.3390/microorganisms9071381|issn=2076-2607|pmc=8304202|pmid=34202088}}</ref> | ||
Laboratory findings - No specific findings; Cytopenias | Laboratory findings - No specific findings; Cytopenias | ||
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*Most are nasal type involving the upper aerodigestive tract | *Most are nasal type involving the upper aerodigestive tract | ||
*Extranasal type may involve skin, testis, and gastrointestinal tract<ref name=":0" />. | *Extranasal type may involve skin, testis, and gastrointestinal tract<ref name=":0" />. | ||
*Bone marrow involvement is uncommon<ref name=":21" />. | *Bone marrow involvement is uncommon<ref name=":21">{{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>. | ||
The <u>morphologic features</u> of this disease are detailed below: | The <u>morphologic features</u> of this disease are detailed below: | ||
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*Loss of mucosal glands. | *Loss of mucosal glands. | ||
*Angiocentric and angiodestructive growth pattern with coagulative necrosis. | *Angiocentric and angiodestructive growth pattern with coagulative necrosis. | ||
*Usually see apoptotic cells and mitotic figures | *Usually see apoptotic cells and mitotic figures[[File:IHC NKTCL.png|thumb|Extranodal NK T-cell lymphoma stained with CD2 (top left), CD56 (red chromogen; top right), EBER in-situ hybridization (bottom left) and TIA1 (bottom right).]] | ||
Pitfalls: | Pitfalls: | ||
*Mucosal ulceration and superimposed inflammation can mimic an inflammatory process, particularly in less aggressive cases<ref name=":22" />. | *Mucosal ulceration and superimposed inflammation can mimic an inflammatory process, particularly in less aggressive cases<ref name=":22">Devins, K., Schuster, S.J., Caponetti, G.C. ''et al.'' Rare case of low-grade extranodal NK/T-cell lymphoma, nasal type, arising in the setting of chronic rhinosinusitis and harboring a novel N-terminal ''KIT'' mutation. ''Diagn Pathol'' 13, 92 (2018). <nowiki>https://doi.org/10.1186/s13000-018-0765-1</nowiki></ref>. | ||
*Pseudoepitheliomatous hyperplasia of the overlying mucosal epithelium can mimic squamous cell carcinoma<ref name=":23" /><ref name=":24" />. | *Pseudoepitheliomatous hyperplasia of the overlying mucosal epithelium can mimic squamous cell carcinoma<ref name=":23">{{Cite journal|last=Ling|first=Yi-Hong|last2=Zhu|first2=Chong-Mei|last3=Wen|first3=Shi-Hong|last4=Luo|first4=Rong-Zhen|last5=Li|first5=Peng|last6=Cao|first6=Yun|last7=Rao|first7=Hui-Lan|last8=Lin|first8=Su-Xia|last9=Cai|first9=Mu-Yan|date=2015-09|title=Pseudoepitheliomatous hyperplasia mimicking invasive squamous cell carcinoma in extranodal natural killer/T-cell lymphoma: a report of 34 cases|url=https://pubmed.ncbi.nlm.nih.gov/25619876|journal=Histopathology|volume=67|issue=3|pages=404–409|doi=10.1111/his.12656|issn=1365-2559|pmid=25619876}}</ref><ref name=":24">{{Cite journal|last=Xiang|first=Chun-Xiang|last2=Chen|first2=Zi-Hang|last3=Zhao|first3=Sha|last4=Gao|first4=Li-Min|last5=Tao|first5=Qing|last6=Zuo|first6=Zhuo|last7=Liu|first7=Xiao-Yu|last8=Liu|first8=Wei-Ping|date=2019-07|title=Laryngeal Extranodal Nasal-type Natural Killer/T-cell Lymphoma: A Clinicopathologic Study of 31 Cases in China|url=https://pubmed.ncbi.nlm.nih.gov/31045893|journal=The American Journal of Surgical Pathology|volume=43|issue=7|pages=995–1004|doi=10.1097/PAS.0000000000001266|issn=1532-0979|pmid=31045893}}</ref>. | ||
The <u>immunophenotype</u> of this disease is detailed below: | The <u>immunophenotype</u> of this disease is detailed below: | ||
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Positive (majority) - cytoplasmic CD3ε, CD2, CD56, granzyme B, and TIA-1 | Positive (majority) - cytoplasmic CD3ε, CD2, CD56, granzyme B, and TIA-1 | ||
Positive (subset) - TCR αβ/γδ, HLA-DR, CD25, pSTAT3, CXCL13, IRF4/MUM1, CD16, Fas, FasL, MATK, CD30<ref name=":25" /><ref name=":26" /><ref name=":27" /><ref name=":28" /><ref name=":29" /><ref name=":30" /><ref name=":31" /> | Positive (subset) - TCR αβ/γδ, HLA-DR, CD25, pSTAT3, CXCL13, IRF4/MUM1, CD16, Fas, FasL, MATK, CD30<ref name=":25">{{Cite journal|last=Li|first=Shaoying|last2=Feng|first2=Xiaoli|last3=Li|first3=Ting|last4=Zhang|first4=Shuang|last5=Zuo|first5=Zhuang|last6=Lin|first6=Pei|last7=Konoplev|first7=Sergej|last8=Bueso-Ramos|first8=Carlos E.|last9=Vega|first9=Francisco|date=2013-01|title=Extranodal NK/T-cell lymphoma, nasal type: a report of 73 cases at MD Anderson Cancer Center|url=https://pubmed.ncbi.nlm.nih.gov/23232851|journal=The American Journal of Surgical Pathology|volume=37|issue=1|pages=14–23|doi=10.1097/PAS.0b013e31826731b5|issn=1532-0979|pmid=23232851}}</ref><ref name=":26">{{Cite journal|last=Jhuang|first=Jie-Yang|last2=Chang|first2=Sheng-Tsung|last3=Weng|first3=Shih-Feng|last4=Pan|first4=Shien-Tung|last5=Chu|first5=Pei-Yi|last6=Hsieh|first6=Pin-Pen|last7=Wei|first7=Chih-Hsin|last8=Chou|first8=Shih-Cheng|last9=Koo|first9=Chiew-Loon|date=2015-02|title=Extranodal natural killer/T-cell lymphoma, nasal type in Taiwan: a relatively higher frequency of T-cell lineage and poor survival for extranasal tumors|url=https://pubmed.ncbi.nlm.nih.gov/25554090|journal=Human Pathology|volume=46|issue=2|pages=313–321|doi=10.1016/j.humpath.2014.11.008|issn=1532-8392|pmid=25554090}}</ref><ref name=":27">{{Cite journal|last=Pongpruttipan|first=Tawatchai|last2=Sukpanichnant|first2=Sanya|last3=Assanasen|first3=Thamathorn|last4=Wannakrairot|first4=Pongsak|last5=Boonsakan|first5=Paisarn|last6=Kanoksil|first6=Wasana|last7=Kayasut|first7=Kanita|last8=Mitarnun|first8=Winyou|last9=Khuhapinant|first9=Archrob|date=2012-04|title=Extranodal NK/T-cell lymphoma, nasal type, includes cases of natural killer cell and αβ, γδ, and αβ/γδ T-cell origin: a comprehensive clinicopathologic and phenotypic study|url=https://pubmed.ncbi.nlm.nih.gov/22314189|journal=The American Journal of Surgical Pathology|volume=36|issue=4|pages=481–499|doi=10.1097/PAS.0b013e31824433d8|issn=1532-0979|pmid=22314189}}</ref><ref name=":28">{{Cite journal|last=Jaffe|first=E. S.|last2=Chan|first2=J. K.|last3=Su|first3=I. J.|last4=Frizzera|first4=G.|last5=Mori|first5=S.|last6=Feller|first6=A. C.|last7=Ho|first7=F. C.|date=1996-01|title=Report of the Workshop on Nasal and Related Extranodal Angiocentric T/Natural Killer Cell Lymphomas. Definitions, differential diagnosis, and epidemiology|url=https://pubmed.ncbi.nlm.nih.gov/8540601|journal=The American Journal of Surgical Pathology|volume=20|issue=1|pages=103–111|doi=10.1097/00000478-199601000-00012|issn=0147-5185|pmid=8540601}}</ref><ref name=":29">{{Cite journal|last=Ohshima|first=K.|last2=Suzumiya|first2=J.|last3=Shimazaki|first3=K.|last4=Kato|first4=A.|last5=Tanaka|first5=T.|last6=Kanda|first6=M.|last7=Kikuchi|first7=M.|date=1997-11|title=Nasal T/NK cell lymphomas commonly express perforin and Fas ligand: important mediators of tissue damage|url=https://pubmed.ncbi.nlm.nih.gov/9416485|journal=Histopathology|volume=31|issue=5|pages=444–450|doi=10.1046/j.1365-2559.1997.2880887.x|issn=0309-0167|pmid=9416485}}</ref><ref name=":30">{{Cite journal|last=Takata|first=Katsuyoshi|last2=Hong|first2=Min-Eui|last3=Sitthinamsuwan|first3=Panitta|last4=Loong|first4=Florence|last5=Tan|first5=Soo-Yong|last6=Liau|first6=Jau-Yu|last7=Hsieh|first7=Pin-Pen|last8=Ng|first8=Siok-Bian|last9=Yang|first9=Sheau-Fang|date=2015-01|title=Primary cutaneous NK/T-cell lymphoma, nasal type and CD56-positive peripheral T-cell lymphoma: a cellular lineage and clinicopathologic study of 60 patients from Asia|url=https://pubmed.ncbi.nlm.nih.gov/25188863|journal=The American Journal of Surgical Pathology|volume=39|issue=1|pages=1–12|doi=10.1097/PAS.0000000000000312|issn=1532-0979|pmid=25188863}}</ref><ref name=":31">{{Cite journal|last=Kuo|first=Tseng-Tong|last2=Shih|first2=Lee-Yung|last3=Tsang|first3=Ngan-Ming|date=2004-10|title=Nasal NK/T cell lymphoma in Taiwan: a clinicopathologic study of 22 cases, with analysis of histologic subtypes, Epstein-Barr virus LMP-1 gene association, and treatment modalities|url=https://pubmed.ncbi.nlm.nih.gov/15494863|journal=International Journal of Surgical Pathology|volume=12|issue=4|pages=375–387|doi=10.1177/106689690401200410|issn=1066-8969|pmid=15494863}}</ref> | ||
Negative (universal) - CD4, CD8 | Negative (universal) - CD4, CD8 | ||