Abstract:Objective:To investigate the clinical features, treatments and prognostic factors of rhabdomyosarcoma in nasal cavity and paranasal sinus. Methods:Clinical data of 15 patients with rhabdomyosarcoma of the nasal cavity and paranasal sinuses admitted to the Second Hospital of Shanxi Medical University from January 2012 to January 2022 were retrospectively analyzed. SPSS 26.0 statistical software was used for descriptive statistical analysis. Kaplan-Meier method was used to evaluate the relationship between clinical data, pathological features, tumor stage and prognosis. Results:Most of the 15 patients with RMS were adolescents. 12 cases were ERMS, 3 cases were ARMS. 1 case received chemotherapy alone. 3 patients received operation and postoperative chemotherapy. 7 patients received surgery and postoperative CRT. 4 patients received preoperative chemotherapy and surgery and postoperative CRT. Follow-up lasted from 3 months to 113 months. After treatments, 6 cases had no recurrence, 4 cases had locoregional recurrence and 5 cases systemic recurrence. 8 patients survived and 7 died. Survival analysis showed that the overall survival rates for 1 year and 5 years were calculated as 93.3% and 43.6%. Pathological type, presence or absence of regional lymph node metastasis, presence or absence of distant metastasis, treatment method, IRS stage were correlated with the prognosis of nasal and paranasal sinus RMS(P<0.05). Conclusions:RMS in the nasal cavity and paranasal sinus is rare in clinic, easily invading the orbit and skull base, with high recurrence rate and mortality. It is easy to invade the orbit and skull base, with high recurrence rate and mortality. Multiple Disciplinary Team is an effective treatment approach. Operation combined with adjuvant chemoradiotherapy can reduce the recurrence rate and improve the prognosis. To improve the clinical and pathological understanding of nasal and paranasal sarcoma, to conduct molecular and genomic studies, and to find new therapeutic targets, it is expected to improve the quality of life and survival rate of RMS patients.