Overview of Peripheral Nerve Sheath Tumors

Peripheral nerve sheath tumors are tumors that originate from peripheral nerve sheath cells. They may involve the perineurium, the epineurium, or Schwann cells. The 2021 edition of the World Health Organization Classification of Tumors of the Central Nervous System lists six distinct entities of peripheral nerve sheath tumors. Neurofibromas, schwannomas, perineuriomas, and hybrid tumors are benign lesions that may occur sporadically or in the context of genetic diseases. Phacomatoses that are typically associated with these kinds of tumors are neurofibromatosis type 1, nf2-related schwannomatosis, and non-nf2-schwannomatosis. Malignant peripheral nerve sheath tumors and malignant melanocytic nerve sheath tumors are very rare entities with poor prognosis. In this chapter, we give an overview of morphological features, epidemiology, and treatment options for all these types of peripheral nerve sheath tumors.