Treatment, outcomes, and demographics in sinonasal sarcoma: a systematic review of the literature

Background Sarcomas comprise a diverse group of soft tissue mesenchymal malignancies. The sinuses and nasal region are a relatively rare site of sarcomas. Methods Retrospective review of the literature on sinonasal sarcomas from 1987-2017. Data were analyzed for demographics, treatment type, stage, and histopathologic type. Kaplan-Meier analysis was used to assess and compare survival. Results A total of 198 cases of sinonasal sarcoma were identified and analyzed. The median age at diagnosis was 39 years. Overall 5-, 10-, and 20-year survival was 61.3%, 58.9%, and 49.1%, respectively, and disease-free 5-, 10-, and 20-year survival was 53.2%, 49.1%, and 38.3%, respectively. Lymph node metastasis was present at diagnosis in 3.0% of cases, and distant metastasis was present in 3.5% of cases. On univariate analysis T stage, overall stage, treatment type, histopathologic subtype, and presence of distant metastasis significantly affected survival. On multivariate analysis overall stage alone significantly predicted overall survival. Open vs. endoscopic surgery, total radiation dose, and presence of neck metastasis did not significantly affect survival. Combined modality treatment was associated with higher survival rates than single modality therapy. Conclusions Sinonasal sarcoma is a relatively rare malignancy. Lower T and overall stage, lack of distant metastasis, and multimodality therapy were associated with improved survival. Certain histopathologic subtypes were associated with poorer survival.