Extranodal nasal NK/T cell lymphoma

Definition

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.

Also known as angiocentric T-cell lymphoma, Extranodal NK/T lymphoma-nasal, Extranodal NK/T-cell lymphoma, nasal type, lethal midline granuloma, nasal T/natural killer-cell lymphoma, nasal type Extranodal NK/T-cell lymphoma, NK/T-cell lymphoma, NKTCL, reticulosis, malignant — per MONDO

Also identified as

• DOID 0080797 per MONDO
• ICD10CM C86.0 per MONDO
• NCIT C4684 per MONDO
• Orphanet 86879 per MONDO
• UMLS C0392788 per MONDO

Affected Organs