A Fatal Ulceration of the Palate

Abstract

Palatal necrosis seen clinically often has a differential diagnosis, such as necrotizing sialometaplasia, extranodal natural killer/T-cell lymphoma (ENKL), primary adenocarcinoma, squamous cell carcinoma, subacute necrotizing sialadenitis (SANS), and secondary syphilis. This case report will discuss and explore the unique disease of extranodal natural killer/T-cell lymphoma (ENKL), formerly known as midline lethal granuloma. ENKL is a rare mature and aggressive neoplasm that accounts for less than 1% of cutaneous lymphomas.2 It is more common in males than females, appears as an ulcerated nodule, and median survival rate is usually between 6-30 months.2ENKL is strongly associated with Epstein Barr Virus and usually seen in Asia and South and Central America with rarity in North America.1 Clinically, rapidly progressive inflammation and necrosis are observed and, on histology, CD56 and CD3 are expressed on malignant cells.1 Prognosis is generally poor, manifests mostly in the nasal and oral region, but can also be seen in dermis, intestines and testis.1,2The authors report a case of a 36-year-old Hispanic male presenting to the emergency room with a 3-month history of an ulcerated palate, which has progressively worsened. Excisional biopsy was completed that returned positive for ENKL.