sinonasal rhabdomyosarcoma
Definition: Rhabdomyosarcoma of the nasal cavities and paranasal sinuses.
Rhabdomyosarcoma is an aggressive malignant tumor often developing in the head and neck in children. In the sinonasal region, rhabdomyosarcoma constitutes a clinically important group because of the difficulty of surgical resection and its generally poor prognosis.
Sinonasal rhabdomyosarcoma is predominantly of the alveolar subtype and frequently shows clear cells. (17478770#)
Synopsis
sinonasal alveolar rhabdomyosarcoma (17478770, 16959712)
- morphological alveolar subtype (33%)
- poorly differentiated, forming a solid alveolar pattern
- PAX3/FKHR chromosomal translocation (4/4)
sinonasal embryonal rhabdomyosarcoma (rare)
References
Ahmed AA, Tsokos M. Sinonasal rhabdomyosarcoma in children and young adults. Int J Surg Pathol. 2007 Apr;15(2):160-5. PMID: 17478770
Manucha V, Castellani R, Sun CC. Alveolar rhabdomyosarcoma of the paranasal sinuses in a 57-year-old woman with 1:16 translocation. Int J Surg Pathol. 2006 Jul;14(3):238-42. PMID: 16959712
Callender TA, Weber RS, Janjan N, Benjamin R, Zaher M, Wolf P, el-Naggar A. Rhabdomyosarcoma of the nose and paranasal sinuses in adults and children. Otolaryngol Head Neck Surg. 1995 Feb;112(2):252-7. PMID: 7838548