Retroperitoneal sarcomas are rare tumors, 25% of them are leiomyosarcomas. On the other hand, 19% of leiomyosarcomas are located in the retroperitoneum. Generally, the diagnosis is made at late stage due to poor symptomatology. Computed tomodensitometry is the most reliable diagnostic procedure. However, the final diagnosis is always obtained after histologic examination. Complete surgical excision is the best treatment. In fact, survival depends on histologic grading and resectability. The roles of radiation therapy and chemotherapy are not yet established.
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