Primary Retroperitoneal Sarcomas

Abstract

Soft tissue sarcomas are rare tumors, 10-20% of which are located in the retroperitoneum. Primary retroperitoneal sarcoma should be considered in patients presenting with abdominal pain, discomfort, or palpable abdominal mass, discovered on physical examination or as incidental findings in CT, US, or MRI. All patients presenting retroperitoneal sarcoma with the possibility of resection should undergo laparotomy, and the surgical plan should include complete resection of the tumor and adjacent infiltrated organs and structures. Chemotherapy shows discouraging results, however preoperative radioterapy may benefit the patient. Retroperitoneal sarcoma tends to present local recurrence, so postoperative follow-up is necessary with detailed clinical history, physical examination, chest X-ray, and abdominal and pelvic CT, and new surgical intervention when possible if recurrence is confirmed. Overall 5-year survival is 40-50%, and the most important prognostic factors are tumor size, degree of differentiation, radical or palliative resection, need for intra-operative blood transfusion, and re-resection even in palliative cases.