Abstract
Simple SummaryThe management of retroperitoneal sarcomas can be challenging due to the variety of their presentation, histopathological types, and behaviours. This literature review provides a comprehensive and practical overview of the management of retroperitoneal sarcomas, focusing on diagnostic challenges, prognostic factors, multidisciplinary aspects of treatment and new research perspectives.Retroperitoneal sarcomas (RPS) are rare cancers whose management can be challenging due to various presentation patterns, multiple organ involvement, and a high local and distant recurrence rate. Histopathology and prognostic factors analysis are essential to predict the behaviour of the disease and plan the best therapeutic strategy. To date, surgery is still the main therapeutic option that guarantees a chance of cure from the primary disease. While chemotherapy and radiotherapy seem to be good options for controlling metastatic and recurrent irresectable disease, their role in the treatment of primary RPS remains unclear. This literature review aims to provide a comprehensive overview of the multidisciplinary aspects of RPS management in high-volume centres, summarising the diagnostic path, the prognostic factors, and the most suitable therapeutic options.
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