Extranodal NK/T-cell Lymphoma, Nasal Type: Report of a Case and Review of the South African Literature

Abstract

A 76-year-old African female was referred to the Ear, Nose and Throat (ENT) clinic at Tygerberg hospital in Cape Town, South Africa, with history of a progressively enlarging painful right-sided nasal mass with nasal obstruction. Her medical history was significant for controlled hypertension and type 2 diabetes mellitus. Clinical examination revealed swelling of the right nasolabial region with associated cellulitis and a necrotic right nasal mass with crusting (Figure 1). Flexible endoscopy of the left nasal cavity showed septal perforation. Haematological tests revealed anaemia and leucocytosis with elevated levels of urea, creatinine, c-reactive protein and erythrocyte sedimentation rate (ESR). A surgical biopsy of the nasal mass was performed under local anaesthesia. A pus swab was also obtained for microbiological culture which identified Staphylococcus aureus and Streptococcus gordonnii. Subsequently treatment with the antibiotic, Clindamycin, was initiated. The patient was then discharged from the hospital, pending histology results. Histological examination of the biopsy obtained under local anaesthesia showed a small specimen with fragmentation artifacts and 
necrosis. A repeat biopsy was recommended. On follow-up, the patient’s condition had worsened. She presented with delirium, dehydration and a significant increase in the size of the nasal mass. The patient was subsequently taken to the operating theatre. Intraoperative exploration of the right nasal cavity disclosed a foul smelling necrotic tissue involving the anterior third of the nasal floor and septum, with extension into the oral cavity and destruction of the anterior maxillary alveolar cortex (Figure 2). Given the clinical history of diabetes mellitus, an invasive fungal infection (Mucormycosis) was highly suspected. However, biopsy from the right nasal region showed superficial mucosa with ulceration and dense chronic inflammation with no evidence of fungal organisms. Further laboratory investigations were undertaken, including antineutrophil cytoplasmic antibodies (ANCA) test, serum angiotensin-converting enzyme (ACE) test, and syphilis serology, to rule out the destructive midface lesions, polyangiitis with granulomatosis (Wegener’s granulomatosis), sarcoidosis and syphilis respectively. All test results were negative.