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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
- ICD-11: 2A90.6
- OMIM: -
- UMLS: C0392788
- MeSH: D054391
- GARD: -
- MedDRA: 10065855
A summary on this disease is available in Español (2015) Italiano (2015) Nederlands (2015)
Detailed information
Disease review articles
- Review article
- English (2013) - Orphanet J Rare Dis
Clinical Outcome Assessment (COA)
- Patient-Centered Outcome Measures (PCOMs)
- English (2023) - PROQOLIDTM
produced/endorsed by ERN(s) 
produced/endorsed by FSMR(s)Additional information
Further information on this disease
Patient-centred resources for this disease
Research activities on this disease
The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.