A 45-year-old man presents with progressive destruction of the nasal septum, crusting, foul discharge, and perforation of the hard palate. Biopsy shows polymorphic infiltrate with angiocentricity and angioinvasion, atypical lymphoid cells positive for CD56 and cytoplasmic CD3, with EBV detected by in situ hybridisation. The most likely diagnosis is:
- A Granulomatosis with polyangiitis
- B Sinonasal squamous cell carcinoma
- C Extranodal NK/T-cell lymphoma, nasal type ✓
- D Rhinoscleroma
Correct answer: C. Extranodal NK/T-cell lymphoma, nasal type
Explanation
Extranodal NK/T-cell lymphoma, nasal type, historically called lethal midline granuloma, produces aggressive midline facial destruction with septal and palatal perforation. The combination of CD56 positivity, cytoplasmic CD3, angioinvasion, and strong Epstein-Barr virus association is diagnostic. Granulomatosis with polyangiitis also destroys the septum but shows necrotising granulomas and c-ANCA positivity rather than EBV-positive atypical lymphocytes. Rhinoscleroma is a Klebsiella-driven chronic granuloma with Mikulicz cells.
Reference: Robbins and Cotran Pathologic Basis of Disease, 10th ed.
High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP
Written and medically reviewed by the StethoPrep medical team.