Giant retroperitoneal sarcoma: a case report and review of the management of retroperitoneal sarcomas.

A 42-year-old man presented with lower abdominal pain and a vague abdominal mass. He underwent resection of a well-differentiated liposarcoma arising from his retroperitoneum measuring 50 cm and weighing 11.7 kg (25.8 lb). This is the second largest retroperitoneal soft-tissue sarcoma (RSTS) that has been reported. Over the last 15 years 1123 patients with RSTS in 25 series have been reported with a mean tumor size of 15.7 cm. RSTS represents 0.10 to 0.15 per cent of all malignancies but 45 per cent of all retroperitoneal tumors. Diagnosis and treatment of RSTS can be extremely challenging for a general surgeon. Symptoms are nonspecific and may occur only after the tumor is very large. Abdominal discomfort is the presenting complaint in 60 to 70 per cent of patients and palpable mass in 70 to 80 per cent. Treatment of RSTS remains surgical. Multiple trials of chemotherapy and radiation therapy show no survival benefit. The only successful treatment of this tumor is complete excision; 51.4 per cent of tumors can be completely excised, and 50.2 per cent of these excisions include adjacent organs. Long-term prognosis without complete excision is grim with average 5- and 10-year survival rates of 16.7 and 8.0 per cent. With aggressive surgical therapy survival is increased to 58.0 and 39.6 per cent.