Charts and slides of 47 patients with primary retroperitoneal sarcomas (excluding pediatric rhabdomyosarcoma) were reviewed to determine clinical presentation, histologic features, extent of surgical resection, operative morbidity and mortality, use of radiation and/or chemotherapy, and survival data. Most patients presented with pain and a palpable mass. Leiomyosarcomas and liposarcomas were the most common tumors. Eighteen of the 47 patients (38%) had complete tumor excision; 68% required resection of adjacent organs. Operative morbidity was 33% with no mortality. After complete resection, the disease-free 5-year survival was 50% and the overall survival was 70% at 5 years; 10-year disease-free survival was 25% with an overall 58% survival at 10 years. Eleven patients (61%) developed recurrent disease with a median interval of 5 years following complete excision. Six patients received adjuvant radiation and/or chemotherapy with four remaining disease-free from 46 to 61 months. Eighteen patients underwent partial excision of tumor and 11 patients underwent biopsy only; these groups had similar survival curves with only 4% alive at 5 years. Their operative morbidity was 18% and mortality was 7%; median time to clinical evidence of tumor progression was 12 months. Sixty per cent of these patients received therapeutic radiation and/or chemotherapy, but their survival was the same as those undergoing surgery alone. These data emphasize the importance of an aggressive surgical approach in the treatment of retroperitoneal sarcomas. Complete tumor resection and total excision of recurrences will allow many patients long-term survival.
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