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- Fernando López, María Costales, Blanca Vivanco, Manuel F Fresno, Carlos Suárez, and José L Llorente.
- Department of Otolaryngology, Hospital Universitario Central de Asturias, Instituto Universitario de Oncología del Principado de Asturias, Oviedo, Asturias, Spain. flopez_1981@yahoo.es
- Auris Nasus Larynx. 2013 Dec 1; 40 (6): 573-6.
AbstractDesmoplastic small round cell tumor (DSRCT) is a rare malignancy with poor prognosis that generally involves the peritoneum. Only rare cases occur outside the abdomen. Its diagnosis can be achieved only by immunohistochemistry and cytogenetic studies. We describe a case of a 61-year-old man referred to our department with a primary sinonasal tumor. The DSRCT diagnosis was confirmed by the presence of a polyphenotypic immunoprofile (positive for cytokeratin, desmin, and neuronspecific enolase) and the characteristic EWS-WT1 gene fusion resulting from the t(11;22)(p13;q12) reciprocal translocation. This reported case of DSRCT draws attention to the importance of including DSRCT in the differential diagnosis of sinonasal tumors. Copyright © 2013 Elsevier Ireland Ltd. All rights reserved.
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