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Extranodal nasal NK/T cell lymphoma

MedGen UID:
140278
Concept ID:
C0392788
Neoplastic Process
Synonyms: Extranodal NK T Cell Lymphoma, Nasal; Extranodal NK T Cell Lymphoma, Nasal and Nasal Type; Extranodal NK T Cell Lymphoma, Nasal Type; Extranodal NK-T-Cell Lymphoma, Nasal; Extranodal NK-T-Cell Lymphoma, Nasal and Nasal-Type; Extranodal NK-T-Cell Lymphoma, Nasal Type
SNOMED CT: Extranodal NK/T-cell lymphoma, nasal type (414166008); Angiocentric T-cell lymphoma (414166008); T/NK-cell lymphoma (414166008); Extranodal natural killer/T-cell lymphoma, nasal type (414166008)
Modes of inheritance:
Non-Mendelian inheritance
MedGen UID:
109109
Concept ID:
C0600599
Genetic Function
Source: Orphanet
A mode of inheritance that depends on genetic determinants in more than one gene.
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0019472
Orphanet: ORPHA86879

Definition

Extranodal nasal NK/T cell lymphoma (NKTCL) is a rare, malignant neoplasm mainly affecting men in the fifth decade of life, that usually arises in the nose, paranasal sinuses, orbits or upper airway, and that can present with a nasal mass, nasal bleeding, nasal obstruction, palate perforation (i.e. midline perforation of the hard palate), and mid-facial and/or upper airway destructive lesions. In advanced disease stages, which are associated with a poor prognosis, NKTCL may disseminate to other organs. A few cases of NKTCL presenting primarily in the lymph nodes have also been described. [from ORDO]

Professional guidelines

PubMed

Yamaguchi M, Oguchi M, Suzuki R
Best Pract Res Clin Haematol 2018 Sep;31(3):315-321. Epub 2018 Jul 3 doi: 10.1016/j.beha.2018.07.002. PMID: 30213402
Yamaguchi M, Miyazaki K
J Clin Exp Hematop 2017 Dec 27;57(3):98-108. Epub 2017 Jul 6 doi: 10.3960/jslrt.17018. PMID: 28679966Free PMC Article
Tse E, Kwong YL
J Hematol Oncol 2017 Apr 14;10(1):85. doi: 10.1186/s13045-017-0452-9. PMID: 28410601Free PMC Article

Curated

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), T-Cell Lymphomas, 2024

Recent clinical studies

Etiology

Qi F, Zhou W, Xie Y, Sun Y, Wu M, Chai Y, Chen B, Lin N, Liu W, Ding N, Li Y, Dong M, Song Y, Zhu J
Aging (Albany NY) 2022 Nov 1;14(21):8729-8744. doi: 10.18632/aging.204355. PMID: 36326691Free PMC Article
Feng Y, Feng X, Jing C, Yu X, Zheng Y, Xu C
Sci Rep 2022 Jan 7;12(1):36. doi: 10.1038/s41598-021-02515-5. PMID: 34996890Free PMC Article
Zhang X, Wu J, Lin D
J Biomed Nanotechnol 2021 Mar 1;17(3):487-500. doi: 10.1166/jbn.2021.3048. PMID: 33875083
Kanitsap N, Warnnissorn N
J Med Assoc Thai 2010 Dec;93 Suppl 7:S294-8. PMID: 21294428

Diagnosis

Feng Y, Feng X, Jing C, Yu X, Zheng Y, Xu C
Sci Rep 2022 Jan 7;12(1):36. doi: 10.1038/s41598-021-02515-5. PMID: 34996890Free PMC Article
Zhen Z, Huang H, Lin T, Li Z, Zhen X, Xia Z, Zhu J, Lu S, Sun F, Wang J, Huang J, Sun X
Pediatr Blood Cancer 2021 May;68(5):e28901. Epub 2021 Jan 23 doi: 10.1002/pbc.28901. PMID: 33484107
Pontes HA, Pontes FS, Silva BS, Fonseca FP, Andrade BA, Rizo VH, Romañach MJ, León JE, Almeida OP
Braz Dent J 2013;24(3):284-8. doi: 10.1590/0103-6440201302202. PMID: 23969921
Romero-Guadarrama MB, Aguilar-Martínez E
Acta Cytol 2010 Sep-Oct;54(5 Suppl):993-7. PMID: 21053585

Therapy

Zhen Z, Huang H, Lin T, Li Z, Zhen X, Xia Z, Zhu J, Lu S, Sun F, Wang J, Huang J, Sun X
Pediatr Blood Cancer 2021 May;68(5):e28901. Epub 2021 Jan 23 doi: 10.1002/pbc.28901. PMID: 33484107

Prognosis

Qi F, Zhou W, Xie Y, Sun Y, Wu M, Chai Y, Chen B, Lin N, Liu W, Ding N, Li Y, Dong M, Song Y, Zhu J
Aging (Albany NY) 2022 Nov 1;14(21):8729-8744. doi: 10.18632/aging.204355. PMID: 36326691Free PMC Article
Feng Y, Feng X, Jing C, Yu X, Zheng Y, Xu C
Sci Rep 2022 Jan 7;12(1):36. doi: 10.1038/s41598-021-02515-5. PMID: 34996890Free PMC Article
Pontes HA, Pontes FS, Silva BS, Fonseca FP, Andrade BA, Rizo VH, Romañach MJ, León JE, Almeida OP
Braz Dent J 2013;24(3):284-8. doi: 10.1590/0103-6440201302202. PMID: 23969921
Kanitsap N, Warnnissorn N
J Med Assoc Thai 2010 Dec;93 Suppl 7:S294-8. PMID: 21294428
Romero-Guadarrama MB, Aguilar-Martínez E
Acta Cytol 2010 Sep-Oct;54(5 Suppl):993-7. PMID: 21053585

Clinical prediction guides

Qi F, Zhou W, Xie Y, Sun Y, Wu M, Chai Y, Chen B, Lin N, Liu W, Ding N, Li Y, Dong M, Song Y, Zhu J
Aging (Albany NY) 2022 Nov 1;14(21):8729-8744. doi: 10.18632/aging.204355. PMID: 36326691Free PMC Article

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    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Curated

    • NCCN, 2024
      NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), T-Cell Lymphomas, 2024

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