Peripheral nerve sheath tumor

Last revised by Ashesh Ishwarlal Ranchod on 25 Sep 2023

Peripheral nerve sheath tumors (PNSTs) are a group of primary neurogenic tumors that arise from nerve sheaths outside of the central nervous system. The vast majority are benign, however, malignant transformation is seen particularly in large tumors and those associated with neurofibromatosis type 1 (NF1)

Many peripheral nerve sheath tumors may express somatostatin receptors 6

Their imaging appearances, demographics, treatment and prognosis vary greatly and these are therefore discussed separately.

Peripheral nerve sheath tumors may be classified as follows:

Imaging of a solitary peripheral nerve sheath tumor, in most cases, cannot reliably distinguish between the different histological subtypes, and a presumptive diagnosis must take into account the patient demographics, including pre-existing conditions (such as neurofibromatosis type 1 or neurofibromatosis type 2), the location and size of the tumor, and evidence of rapid growth. As such these are discussed separately in each of the aforementioned tumors. A number of features are, however, shared by localized neurogenic tumors of peripheral nerves.

As a group, localized peripheral nerve sheath tumors demonstrate the following features:

Considerations include 7,8:

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