Sinonasal desmoplastic small round cell tumor: a case report and review of the literature

BACKGROUND: Desmoplastic small round cell tumor (DSRCT) is a rare malignancy with poor prognosis that generally involves the peritoneum. Its diagnosis can be achieved only by immunohistochemistry and cytogenetic studies. CASE PRESENTATION: In the current report, a 55-year-old female was admitted in our hospital for evaluation of right eye epiphora and right nasal intermittent bleeding. Imaging examination revealed a large soft tissue mass in the right nasal cavity and ethmoid sinus. After an explorative surgery, the pathological findings confirmed the presentation of sinonasal DSRCT. Immunohistochemistry and cytogenetic studies confirmed the diagnosis of DSRCT in this patient. Surgical resection, chemotherapy, and radiotherapy was performed, and she died 2 months after operation. CONCLUSION: This reported case draws attention to the importance of novel treatments and including DSRCT in the differential diagnosis of sinonasal tumors.