Epithelioid sarcoma: half a century later

Background and objectives: Epithelioid sarcoma (ES) is an aggressive malignancy scarcely reported on due to its rarity. This study is a review of its traits and features of prognosis and survival by analyzing both the literature and a national cancer database. Methods: Data were acquired from both the Survival, Epidemiology, and End Results database and literature. 1, 5, and 10-year Disease Specific Survival rates and hazard ratios (HR) were determined. Data were split into pre-2000 (<2000) and post-2000 (>2000) groups. Overall survival, recurrence, and metastasis rates were obtained. Results: Ninety hundred and ninety eight and 992 cases of ES were identified from the database and literature, respectively. Age, anatomical site, grade, TNM staging, treatment modality and year of diagnosis were demonstrated to be independent predictors of survival. Overall 5- and 10-year survival were 60.4% and 50.2%, respectively. Overall recurrence and metastasis rates were 63.4% and 40.3%. Using cases diagnosed prior to 2000 as reference, those diagnosed after 2000 had a worse prognosis (HR: 1.55). Conclusions: We report using the largest cohort of ES to date. Despite ES's often dismal prognosis, there are factors associated with better outcomes. A worsening survival over the years warrants further investigation into this sarcoma.