Extranodal nasal NK/T cell lymphoma

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Disease Overview

Extranodal nasal NK/T cell lymphoma (NKTCL) is a rare type of cancer. The term extranodal is used because this form of lymphoma is found outside of the traditional lymph node groupings. It mainly affects men around 50 years of age, and usually arises in the nose, paranasal sinuses (paranasal sinuses are cavities (spaces) or small tunnels. located around or near the nose), orbits or upper airway, and that can present with a nasal mass, nasal bleeding, nasal obstruction,  a hole in the palate, and mid-facial and/or upper airway destructive lesions. In advanced disease stages, which are associated with a poor prognosis, NKTCL may affect other organs, but usually without enlarged nodes in the body.[13354][13355][13356] The treatment depends on the extent of disease and usually involves radiotherapy and chemotherapy.[13357]


Synonyms

  • NK/T-cell lymphoma
  • Angiocentric T-cell lymphoma
  • Lethal midline granuloma
  • NKTCL
  • Nasal T/natural killer-cell lymphoma

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National Organization for Rare Disorders