Schwannomas are tumors of neural origin. They represent 1% of all the tumors of the retroperitoneum. The majority are benign. However, up to 18% are malignant, and frequently with a low-grade differentiation in their histology. A 46-year-old female with neurofibromatosis type 1 who had right knee pain with irradiation to the ipsilateral leg was described. The diagnostic approach included lumbar magnetic resonance imaging showing a retroperitoneal tumor from L2 to L3. Despite the great development of therapeutic strategies, surgical excision seems to be the only one with curative potential. However, few patients are candidates for this treatment. In our case, we gave the patient a multidisciplinary treatment which leads to a good oncological outcome. Malignant degeneration of schwannomas is not uncommon. We propose the multidisciplinary approach for the diagnosis, the surgical procedure as well as follow-up for better outcomes.
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