There is paucity of randomized controlled data on the role of systemic therapy in retroperitoneal sarcomas. The type of systemic therapy used is guided by the histological subtype. The majority of retroperitoneal sarcomas comprising liposarcomas and leiomyosarcomas are by and large chemotherapy insensitive. There is an urgent need for more efficacious systemic therapies in the management of early and advanced stage retroperitoneal sarcomas.
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