Malignant peripheral nerve sheath tumors (MPNSTs) are soft-tissue sarcomas that surround peripheral nerves and occur either sporadically or more often in association with neurofibromatosis type 1 (NF1, von Recklinghausen disease), an inherited disorder caused by germline mutations of the NF1 gene [1,2]. This evidence concerns the gene NF1 and malignant peripheral nerve sheath tumor.