Clinical Management and Risk Stratification of Retroperitoneal Sarcoma with a Focus on Locally Recurrent Disease

Opinion Statement Retroperitoneal sarcomas are a group of rare malignancies comprised of several histological types, each carrying a unique risk of local recurrence and metastatic potential. In primary disease, surgery remains the mainstay of treatment and is the best opportunity for cure. In recurrent disease, treatment algorithms are less well defined. Surgery, radiation, systemic therapy, and in select cases, observation can be considered for management of recurrent disease. Histologic type drives a significant portion of clinical decision-making but incorporating all relevant clinicopathological data is important. To guide selection and timing of treatment, patients should undergo risk stratification. Nomograms and risk calculators are available to facilitate this. Experience and clinical judgement remain paramount. In the future, deeper molecular and radiologic characterization may further optimize decision-making in these challenging cases.