Background: Low grade fibromyxoid sarcoma or tumor Evans is a variety of soft tissue sarcoma that represents 1% of all malignancies. More common in limbs and trunk (50%), we present 3 new cases of retroperitoneal reviewing their characteristics and performing a literature review.

Clinical Case: In the retroperitoneal location highlights the poor specificity of clinical symptoms, demonstrating, according to their growth and size, as an abdominal tumor, usually painless, or by compression and/or invasion of nearby structures. In cases that present the most important finding was the presence of a palpable abdominal tumor without other symptoms despite remarkable infiltration of other organs that should be included in surgical resection.

Conclusions: We emphasize the difficulty of correct diagnosis preoperatively because preoperative studies are inconclusive and only the histological and immuno-histo-typing chemistry allow precise identification.

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