title: Sinonasal desmoplastic small round cell tumor.
abstract: Desmoplastic 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.
authors: Department of Otolaryngology, Hospital Universitario Central de Asturias, Instituto Universitario de Oncología del Principado de Asturias, Oviedo, Asturias, Spain. flopez_1981@yahoo.es
authors: Fernando
authors: F
authors: López
authors: María
authors: M
authors: Costales
authors: Blanca
authors: B
authors: Vivanco
authors: Manuel F
authors: MF
authors: Fresno
authors: Carlos
authors: C
authors: Suárez
authors: José L
authors: JL
authors: Llorente
mesh_terms: D058405:Desmoplastic Small Round Cell Tumor / Q000175:diagnosis* / Q000473:pathology; D003937:Diagnosis, Differential; D050939:Gene Fusion; D006801:Humans; D007150:Immunohistochemistry; D017404:In Situ Hybridization, Fluorescence; D008297:Male; D008875:Middle Aged; D010255:Paranasal Sinus Neoplasms / Q000175:diagnosis* / Q000473:pathology
keywords: Desmoplastic small round cell tumor; EWS/WT1 gene fusion; Immunohistochemistry; Molecular analysis; Sinonasal tumor