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Start free with EleplanExtranodal nasal NK/T cell lymphoma
ORPHA:86879Disease
Also called Angiocentric T-cell lymphoma · Lethal midline granuloma · NK/T-cell lymphoma · NKTCL · Nasal T/natural killer-cell lymphoma
What it is
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.
Key facts
- Prevalence
- 1-9 / 100 000 (annual incidence, France)
- Age of onset
- Adult
- Inheritance
- Multigenic/multifactorial, Not applicable
- Classified as
- Disease
Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.
ICD-10 codes
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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