Background: Retroperitoneal sarcoma (RPS) is considered a disease with poor prognosis partly because of the difficulty with diagnosis at an early stage. This review assesses the current best practice principles for RPS and finds evidence suggesting a better outlook for appropriately managed cases. Recommendations are made for improving diagnostic certainty before laparotomy and inappropriate transperitoneal biopsy occur.
Methods: A critical review of the English language literature was conducted using MEDLINE software and searching the terms 'retroperitoneal sarcoma' alone or in combination with 'prognosis', 'surgery' and 'adjuvant therapy'.
Conclusions: Retroperitoneal sarcoma is a rare disease but when appropriately managed the disease-free survival can be improved and may even approach that of extremity soft tissue sarcoma. One of the greatest barriers to improving outcome is the misinterpretation of clinical signs and an over-reliance on ultrasound diagnosis in pelvic presentations, or misinterpretation of clinical signs and/or computer tomography (CT) scans in abdominal masses. Physicians referring patients with a retroperitoneal mass should consider more frequently the less common differential diagnoses of an abdominopelvic mass including retroperitoneal sarcoma. This is especially true in circumstances where there is a circumscribed, predominantly solid tumour, with clinical or radiological signs of vascular or rectal displacement, ureteric obstruction and/or classic renal rotational displacement. The more frequent use of CT scans with intravenous and oral contrast with referral prior to inappropriate transperitoneal biopsy is recommended. In atypical cases where preoperative biopsy is necessary, extraperitoneal routes are preferable. Complete en bloc surgical excision at the first laparotomy is the treatment of choice in RPS. Macroscopic clearance may necessitate resection of adjacent viscera, neurovascular structures or abdominopelvic walls but, if achieved, may lead to long-term survival depending on individual tumour biology.