A 42-year-old man presents with progressive ulcerative destruction of the nasal septum and palate. Biopsy shows a dense infiltrate of medium-sized atypical lymphoid cells invading and destroying blood vessel walls. Immunohistochemistry shows cytoplasmic CD3 positivity, strong CD56, granzyme B, and positive EBER in situ hybridization. What is the most likely diagnosis?
- A Extranodal diffuse large B-cell lymphoma
- B Extranodal NK/T-cell lymphoma, nasal type ✓
- C Angioimmunoblastic T-cell lymphoma
- D Peripheral T-cell lymphoma, not otherwise specified
Correct answer: B. Extranodal NK/T-cell lymphoma, nasal type
Explanation
Extranodal NK/T-cell lymphoma, nasal type, classically destroys midfacial structures, shows angiocentric and angiodestructive growth, and consistently harbors clonal episomal EBV demonstrated by EBER. Its phenotype combines surface CD3 negativity with cytoplasmic CD3 epsilon, strong CD56, and cytotoxic granule proteins. Extranodal DLBCL is CD20 positive and EBV negative in immunocompetent patients, and angioimmunoblastic lymphoma presents as systemic lymphadenopathy rather than localized nasal destruction.
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.