Auris, nasus, larynx, 2013-12, Vol.40 (6), p.573-576
2013
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- Autor(en) / Beteiligte
- Titel
- Sinonasal desmoplastic small round cell tumor
- Ist Teil von
- Auris, nasus, larynx, 2013-12, Vol.40 (6), p.573-576
- Ort / Verlag
- Netherlands: Elsevier Ireland Ltd
- Erscheinungsjahr
- 2013
- Quelle
- MEDLINE
- Beschreibungen/Notizen
- 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.
- Sprache
- Englisch
- Identifikatoren
- ISSN: 0385-8146eISSN: 1879-1476DOI: 10.1016/j.anl.2012.12.006Titel-ID: cdi_proquest_miscellaneous_1420163435
- Format
- –
- Schlagworte
- Desmoplastic small round cell tumor, Desmoplastic Small Round Cell Tumor - diagnosis, Desmoplastic Small Round Cell Tumor - pathology, Diagnosis, Differential, EWS/WT1 gene fusion, Gene Fusion, Humans, Immunohistochemistry, In Situ Hybridization, Fluorescence, Male, Middle Aged, Molecular analysis, Otolaryngology, Paranasal Sinus Neoplasms - diagnosis, Paranasal Sinus Neoplasms - pathology, Sinonasal tumor