PURPOSE: Sinonasal rhabdomyosarcoma (SNRMS) is a rare malignancy which often presents with nasal obstruction, rhinorrhea and epistaxis. It is the most common sarcoma in children. In this study, we analyze the incidence and long-term survival for SNRMS using a national population-based database. METHODS: The United States National Cancer Institute's Surveillance, Epidemiology, and End Results (SEER) registry was utilized to calculate incidence and survival trends for SNRMS between 1973 and 2009. In addition, data were grouped by age, gender, race, and histopathological subtype. RESULTS: A total of 181 cases of SNRMS were analyzed for incidence trends, showing a 1.23:1 female to male ratio. While the overall incidence of SNRMS increased by 1.02% annually over the last 20years, this pattern was not equal amongst gender and racial groups. The incidence in males has increased, while in females incidence has decreased. An increase in incidence was noted in white and "others," but decreased in blacks. Using a total of 314 cases for survival analysis, we found that the rate in the white population has been consistently highest with a 5-year survival of 49.45%, 10- and 20-year survival of 48.81%. Survival rates in cases of embryonal SNRMS were also consistently higher than in cases of alveolar SNRMS. CONCLUSION: Overall incidence of SNRMS is increasing. Histologic subtype and race are important considerations in the long-term prognosis of SNRMS. Future studies will further elucidate gender and race related trends.
PURPOSE:Sinonasal rhabdomyosarcoma (SNRMS) is a rare malignancy which often presents with nasal obstruction, rhinorrhea and epistaxis. It is the most common sarcoma in children. In this study, we analyze the incidence and long-term survival for SNRMS using a national population-based database. METHODS: The United States National Cancer Institute's Surveillance, Epidemiology, and End Results (SEER) registry was utilized to calculate incidence and survival trends for SNRMS between 1973 and 2009. In addition, data were grouped by age, gender, race, and histopathological subtype. RESULTS: A total of 181 cases of SNRMS were analyzed for incidence trends, showing a 1.23:1 female to male ratio. While the overall incidence of SNRMS increased by 1.02% annually over the last 20years, this pattern was not equal amongst gender and racial groups. The incidence in males has increased, while in females incidence has decreased. An increase in incidence was noted in white and "others," but decreased in blacks. Using a total of 314 cases for survival analysis, we found that the rate in the white population has been consistently highest with a 5-year survival of 49.45%, 10- and 20-year survival of 48.81%. Survival rates in cases of embryonal SNRMS were also consistently higher than in cases of alveolar SNRMS. CONCLUSION: Overall incidence of SNRMS is increasing. Histologic subtype and race are important considerations in the long-term prognosis of SNRMS. Future studies will further elucidate gender and race related trends.
Authors: Lester D R Thompson; Vickie Y Jo; Abbas Agaimy; Antonio Llombart-Bosch; Gema Nieto Morales; Isidro Machado; Uta Flucke; Paul E Wakely; Markku Miettinen; Justin A Bishop Journal: Head Neck Pathol Date: 2017-09-05