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Extranodal nasal NK/T cell lymphoma

Disease 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.

ORPHA:86879

Classification level: Disorder
  • Synonym(s):
    • Angiocentric T-cell lymphoma
    • Lethal midline granuloma
    • NK/T-cell lymphoma
    • NKTCL
    • Nasal T/natural killer-cell lymphoma
  • Prevalence: -
  • Inheritance: Multigenic/multifactorial or Not applicable 
  • Age of onset: Adult
  • ICD-10: C86.0
  • OMIM: -
  • UMLS: C0018197  C0392788
  • MeSH: D054391
  • GARD: -
  • MedDRA: 10065855

Detailed information

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