Alveolar Rhabdomyosarcoma of the Paranasal Sinuses in a 57-Year-Old Woman With 1:16 Translocation

Abstract

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.

Comparing Adult and Pediatric Rhabdomyosarcoma in the Surveillance, Epidemiology and End Results Program, 1973 to 2005: An Analysis of 2,600 Patients

“…In pediatric alveolar RMS, for example, translocations involving FKHR are the hallmark of this subtype, with most patients having t(2;13)(q35;q14) or t(1;13)(p36; q14) translocations. 26 Abnormalities of FKHR with or without the typical translocations were identified in adults [27][28][29] but the frequencies of these abnormalities in adults are unknown. Significantly, in our study, alveolar histology was not found to be associated with worse survival than the embryonal subtype when tested using a multivariate model in adults.…”

Embryonal and Alveolar Rhabdomyosarcoma of Parameningeal Sites in Adults: A Report of 13 Cases

“…Most cases of adult sinonasal RMS have been reported in patients less than 35 years of age with a few scattered reports in older patients. [11][12][13]21 In the current study, however, more than 50% of the patients were >35 years old at the time of diagnosis, and overall, 23% of patients were greater than 50 years old. In fact, 1 patient was 86 years old at the time of diagnosis, so whereas RMS is extremely rare in the elderly, the diagnosis should never be excluded without thorough evaluation.…”

“…4 Parameningeal RMSs have been rarely reported in adults, particularly young adults. [5][6][7][8][9][10][11][12][13][14][15][16][17][18][19] We report 13 adult patients, ranging in age from 18 to 86 years, with RMS involving parameningeal sites, predominantly the sinonasal cavity.…”

PAX—FKHR Fusion Genes and AChR-γ in Chinese Patients With Rhabdomyosarcoma: Diagnosis Using Formalin-Fixed Archival Tissues

“…McManus et al 27 reported that up to 11% of ARMSs possessed der(16)t(1;16)(q21;q13). Manucha et al 28 also reported a case of ARMS arising in the ethmoid sinus, which presents 45, XX, −5, −13, der(16)t(1;1)(q21;q13).…”