Malignant peripheral nerve sheath tumor
（重定向自Neurofibrosarcoma）
![](/Images/godic/202501/29/Malignant_peripheral_nerve_sheath_tumour_-_high_mag5407.jpg")
![MPNST](/Images/godic/202501/29/MPNST5407.PNG")
![Autosomal dominant](/Images/godic/202501/29/Autosomal_dominant5407.png")
A malignant peripheral nerve sheath tumor (MPNST) (also known as "malignant schwannoma," "neurofibrosarcoma," and "neurosarcoma") is a form of cancer of the connective tissue surrounding nerves. Given its origin and behavior it is classified as a sarcoma. About half the cases are diagnosed in people with neurofibromatosis; the lifetime risk for an MPNST in patients with neurofibromatosis type 1 is 8-13%. MPNST with rhabdomyoblastomatous component are called malignant triton tumors.