The mechanism of pathogenesis for the MPNST is likely related to second‐hit inactivation of NF1, together with mutations in other genes, such as WNK3 which plays a role in the increase of cell survival in a caspase‐3‐dependent pathway, and EHBP1 which has been implicated in endocytic trafficking. This evidence concerns the gene EHBP1 and malignant peripheral nerve sheath tumor.