Abstract
Retroperitoneal sarcomas are heterogeneous tumours with variable disease biology and outcomes. The prognosis is primarily related to tumour histology and grade as well as the ability to achieve margin negative resection. Surgery involves compartment or contiguous organ resection to achieve the above goal. Careful utilization of neoadjuvant and adjuvant strategies like radiotherapy and/or chemotherapy can lead to improvement in margin status, thereby contributing to better local control and possibly reducing systemic dissemination. Use of targeted therapies has paved newer pathways of treatment integration centred on molecular and genetic targets. The aim of this review is to update the reader on all aspects of retroperitoneal sarcoma management including emphasis on pertinent and landmark trials in this regard.
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