Other Peripheral Nerve Tumors Not Included In The Who Classification System

We have described benign peripheral nerve tumors arising primarily from Schwann cells, in the case of schwannomas and neurofibromas, and perineural cells in the case of perineuri-omas. Other tumors arising from cells normally found in peripheral nerves that will be discussed include mucosal neuroma, neurothekeoma, granule cell tumor, lipoma, vascular tumors, and neuromuscular hamartoma. Peripheral nerve tumors can also originate from cells not normally found in nerves by local invasion, as in the case of desmoid tumors (i.e., from adjacent muscle tissue) or by metastatic spread in the case of primary tumors located at a distance. For example, a variety of primary tumors, such as breast and lung tumors, can metas-tasize to peripheral nerves and their plexi. More rarely, thyroid tumors; osteochondromas; bladder tumors; extracranial meningiomas; and malignant Ewing's tumors, which include Ewing's sarcoma, extraosseous Ewing's sarcoma, primitive neuroecto-dermal tumors (PNETs) or peripheral neuroepithelioma, and Askin's tumor (PNET of the chest wall), have all been found to directly involve peripheral nerves. Masses involving peripheral nerves that mimic tumors include ganglion cyst, Morton's neuroma, and traumatic neuroma. Finally, diseases that can mimic peripheral nerve tumors include inflammatory pseudotumor of the peripheral nerve, one of the Charcot-Marie-Tooth (CMT) disorders, radiation-induced neuropathy, and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Tumors arising from neurons in the PNS include ganglioneu-roma, neuroblastoma, peripheral neuroepithelioma, paragan-glioma, and pheochromocytoma. They are often categorized under PNETs and tumors with nerve cell differentiation. They represent a very diverse group of tumors, often found in a pedi-atric population. Their clinical presentation, evaluation, and treatment are quite different from those associated with peripheral nerve tumors, and therefore this group of tumors is dealt with elsewhere in a more detailed fashion.3,48

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