Abstract
The newly emerging sinonasal carcinomas have demonstrated diverse morphologies and specific molecular mutations along with deviant clinical behavior from conventional counterparts. Also, many sinonasal malignancies turned to be SMARCB1/SMARCA4-deficient. Even with the morphological similarities between poorly differentiated (non) keratinizing sinonasal squamous cell carcinoma (SNSCC) from DEK::AFF2 SNSCC, the two lesions are not distinguishable using the surrogate immunohistochemical marker AFF2 or molecular testing for DEK::AFF2 mutation. We report a rare case of SMARCB1-retained DEK-rearranged papillary non-keratinizing SNSCC in a 53-year-old female, who presented with a polypoid mass corresponding to the left middle turbinate. The tumor and locoregional lymph nodes were surgically resected, followed by adjuvant radiotherapy.