Definition
Peripheral nerve sheath tumors (or PNSTs) are a rare type of soft tissue tumors arising from the cells located in the sheath of peripheral nerves.
Characteristics
- Can be either benign or malignant
- In most cases, tumor cells show Schwann cell differentiation
- Schwann cells are the cells that produce myelin in the CNS
- Associated with neurofibromatosis (NF) type I and II
- NF-I is characterized by the loss of the tumor suppressor gene neurofibromin (chromosome 17)
- NF-II is characterized by the loss of tumor suppressor gene merlin (chromosome 22)
- Café-au-lait macules are a tell-tale sign of