INTRODUCTIONRetroperitoneal sarcomas constitute a rare group of tumors with propensity to grow locally in the retroperitoneal space and less often to spread hematogenously. About 20‐25% of retroperitoneal sarcomas will develop distant metastases resulting in a dismal median overall survival of 13 months. The role of surgery for local control is well established in the management of these tumors while the role of radiotherapy, particularly in the neoadjuvant setting, is currently being addressed in randomized controlled trials. The role of systemic therapy, however, is yet to be defined although systemic therapy has been used both in the adjuvant/neoadjuvant setting as well as in the advanced/metastatic setting in the management of these tumors.The main purpose of systemic treatment in the neoadjuvant setting is to reduce the size of the primary tumor in order to facilitate R0 resection often reducing the need for extensive multiorgan resection. In addition, neoadjuvant systemic therapy is used to control micrometastatic disease, to assess in vivo chemosensitivity and prevent prolonged administration of ineffective treatment. In the adjuvant setting, drug therapy is given to treat micrometastatic disease and ultimately reduce the risk of recurrence. In the advanced setting the aim of systemic therapy is to reduce disease bulk,
Journal of Surgical Oncology – Wiley
Published: Jan 1, 2018
Keywords: ; ; ; ;
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