MPNST (previously known as neurogenic sarcoma, neurofibrosarcoma, or malignant schwannoma) is a relatively rare malignant tumor, accounting for 5–10% of all soft tissue sarcomas with an incidence rate of 0.001% in the general population and 4.6% in patients with NF1 [7]. This evidence concerns the gene NF1 and neurofibrosarcoma.