CASE PRESENTATION: The featured case describes a 40-year-old male who presented with symptoms suggestive for sinusitis. Further diagnostic investigation by the functional endoscopic sinus surgery (FESS) revealed a chronic sinusitis with multiple biopsies showing negative for malignancy, viral and bacterial infections and therefore undiagnosed for sinonasal NK/T-cell lymphoma. Subsequently after a month of surgery, he developed multiple lymph nodes in inguinal where biopsy revealed extranodal NK/T-cell non-Hodgkin lymphoma, high grade but in no time for treatment, he finally succumbed to the illness.
CONCLUSION: The case presented here was initially diagnosed as a chronic sinusitis, not as sinonasal NK/Tcell lymphoma which later developed into extranodal NK/T-cell lymphoma. The prognosis showed improvement for nasal lymphomas but remains poor for disseminated and extranasal lymphomas which are more aggressive with lower survival rate. It is clinically important to differentiate diseases for proper staging and monitoring as they require completely different treatment strategies.
CASE PRESENTATION: A 5-year-old Somalian boy with no known medical illness presented with progressive nasal blockage associated with clear nasal discharge and intermittent spontaneous epistaxis for three months. CT paranasal sinus and neck region revealed poorly enhancing expansile mass in the right maxillary sinus with areas of necrosis within. Initial radiological differential diagnoses were lymphoma and rhabdomyosarcoma. The mass was biopsied and histologically showed diffuse sheets of small round blue cells that was positive to CD99, NSE and vimentin. The muscle and lymphoid markers were negative. Fluorescence in-situ hybridisation (FISH) study revealed the presence of EWSR1 gene rearrangement thus diagnosis of ES was rendered.
CONCLUSIONS: ES of sinonasal tract is a rare entity and its pathological features significantly overlap with others small round blue cells tumour. Demonstration of EWSR1 gene translocation is recommended for the diagnosis of ES particularly at uncommon sites.