A Clinical Summary of PNST in Adults

Primary tumors of the peripheral nerve are rare representing approximately 1% of all cancer, range from benign to highly malignant lesions, including schwannoma, perineurioma, neurofibromas, hybrid nerve sheath tumors intraneural and extra neural lipomas, lipomatosis of nerve (LN) ANNUBP, MMNST, and MPNST. Myxopapillary ependymoma, hemangioblastoma, and paraganglioma are often mentioned as tumors of the central nervous system but they can involve the spinal nerve roots. A variety of sarcomas may be observed in the paraspinal region, nerve and roots, as well as lymphoma; in particular, the diffuse B cell type can arise in peripheral nerves. Lastly, the peripheral nerves can be also infiltrated by a primary carcinoma localized outside the nervous system; in this case, they are called secondary tumor. The chapter is focused on spinal roots, plexus, and peripheral nerves tumors; cranial nerves tumors are not included in this chapter, as well as secondary PNS tumors.