Extranodal Natural Killer/T-cell lymphoma, nasal type: ‘midline lethal granuloma.’ A case report

Extranodal NK/T cell lymphoma, nasal type, is rare, but even more rare in black persons. The purpose of this article is to report a severe case of extranodal NK/T cell lymphoma, nasal type, in an elderly black male.Extranodal natural killer/T cell (NK/T cell) lymphoma, nasal type, is a rare non-Hodgkin lymphoma originating in the nasal cavity or in the paranasal sinuses. It is strongly associated with Epstein-Barr virus (EBV) infection. Its prevalence is higher in countries in South-East Asia and in Central and South America than in Europe and in North America; it occurs in middle-aged persons and affects males more frequently than females [1-5]. Most cases arise from natural killer cells, only a few from cytotoxic T-cells [6,7].Clinically, extranodal NK/T cell lymphoma, nasal type, is characterized by progressive midline facial destruction. Initial signs and symptoms include nasal stuffiness, epistaxis and pain, owing to progressive tumour growth in the nose. As the tumour mass enlarges, invading and destroying structures in the upper anterior aerodigestive tract, it becomes progressively necrotic with a purulent discharge. Signs and symptoms are related to the sites involved. Secondary infection and haemorrhage are not infrequent [5,6]. Metastasis is uncommon [8].As extranodal NK/T cell lymphoma, nasal type, may clinically mimic other destructive disease entities affecting mid-facial structures including other lymphomas, nasopharyngeal squamous cell carcinoma, tertiary syphilis, Wegener granulomatosis and fungal infections, the definitive diagnosis must be based on histopathological, immunological and molecular studies [5].Localised extranodal NK/T cell lymphoma, nasal type usually responds favourably to radiotherapy. As in any neoplasm the best clinical outcome is achieved when treatment is started early in the course of the disease; but once the tumour has invaded, radiotherapy must be supplemented with chemotherapy. Nevertheless, local recurrence occurs in abou