Екстранодальна назальна NK/T-клітинна лімфома
ORPHA:86879· ICD-10 C86.0· Extranodal nasal NK/T cell lymphoma
Визначення(English summary)
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.
- Поширеність
- 1-9 / 100 000
- Успадкування
- Multigenic/multifactorial, Not applicable
- Вік початку
- Adult