Alveolar rhabdomyosarcoma of the paranasal sinuses in a 57-year-old woman with 1:16 translocation.
Alveolar rhabdomyosarcoma is an uncommon tumor. It is a rare malignancy in adults and rarely occurs in paranasal sinuses in those aged older than 50 years. This report describes a locally invasive and destructive alveolar rhabdomyosarcoma arising in the ethmoid sinus of a 57-year-old woman. The small round blue cell tumors are positive for myogenin by immunohistochemistry and have a karyotype of 45, XX, -5, -13, der(16)t(1;1) (q21;q13) by cytogenetic analysis. Fluorescence in situ hybridization demonstrated a complex translocation with break apart of the FKHR region, which supports a diagnosis of alveolar rhabdomyosarcoma. This report characterizes this tumor through microscopic and cyto-genetic analysis and emphasizes the importance of considering rhabdomyosarcoma in the differential diagnosis of small round cell tumors of the head and neck region in the middle-aged adults.
Manucha
Varsha
V
Department of Pathology, University of Maryland Medical Center, Baltimore, Maryland; 20 South Greene Street, Baltimore, MD 21201, USA. varshamanucha@yahoo.com
Castellani
Rudy
R
Sun
Chen Chih
CC
D018288:Carcinoma, Small Cell / Q000175:diagnosis; D002878:Chromosomes, Human, Pair 1; D002885:Chromosomes, Human, Pair 16; D003937:Diagnosis, Differential; D018304:Esthesioneuroblastoma, Olfactory / Q000175:diagnosis; D005260:Female; D006801:Humans; D007621:Karyotyping; D008223:Lymphoma / Q000175:diagnosis; D008545:Melanoma / Q000175:diagnosis; D008875:Middle Aged; D018008:Myogenin / Q000032:analysis; D010255:Paranasal Sinus Neoplasms / Q000737:chemistry / Q000235:genetics / Q000473:pathology*; D018232:Rhabdomyosarcoma, Alveolar / Q000737:chemistry / Q000235:genetics / Q000473:pathology*; D014057:Tomography, X-Ray Computed; D014178:Translocation, Genetic
D018008:Myogenin