Malignant peripheral nerve sheath tumor
| Malignant peripheral nerve sheath tumor | |
|---|---|
| Other names | Malignant schwannoma, Neurofibrosarcoma, and Neurosarcoma |
| Micrograph of malignant peripheral nerve sheath tumour with the typical herringbone pattern. H&E stain. | |
| Specialty | Neuro-oncology |
A malignant peripheral nerve sheath tumor (MPNST) is a form of cancer of the connective tissue surrounding peripheral 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.
The first-line treatment is surgical resection with wide margins. Chemotherapy and often radiotherapy are done as adjuvant and/or neoadjuvant treatment depending upon various risk factors.