Retroperitoneal soft-tissue sarcomas: Prognosis and treatment of primary and recurrent disease

Soft-tissue sarcomas of the retroperitoneum constitute a heterogeneous grou p of tumors with varying histology, potential for complete resection, and p ropensity for recurrent disease-making the development of effective treatme nt difficult and challenging. A retrospective review of 23 patients with re troperitoneal sarcomas from 1985 through 1998 was performed to assess the b iological behavior and clinical outcomes and to identify factors that may i nfluence prognosis and optimize treatment strategy. Liposarcomas were the m ost common pathology (61%); 79 per cent of these were of low grade. Leiomyo sarcomas were the next most common pathology (30%); 43 per cent of these we re of low grade. Low-grade sarcomas overall accounted for 62 per cent of th e total group. Low-grade tumors independent of histologic type exhibited go od prognosis for long-term survival with a median survival of 44 months. In contrast, intermediate- or high-grade tumors were associated with a median survival of only 9 months (P < 0.02). On the other hand, tumor histologic type independent of grade did not have a significant survival difference. C omplete tumor resection was possible in 21 of 23 patients, which gives an o verall resectability rate of 91 per cent. Eight patients (36%) remain disea se-free after initial surgical treatment. However, local recurrence was com mon; this occurred in 11 of 22 patients (50%). Local recurrence, however, d id not preclude long-term survival. Surgical resection of recurrent disease was done in nine patients with a median survival of 91 months (range 24-15 0 months). Three patients had as many as three operations for recurrent dis ease. With subsequent recurrences there was a decrease in interval from app roximately 4 years to 2 years, and 33 per cent of these patients developed tumor dedifferentiation to high grade. An aggressive surgical approach with reoperation can produce prolonged survival in patients with low-grade retr operitoneal sarcoma.