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Definitive diagnosis is made by tumor biopsy. [12] Surgery is the most common method of treating peripheral nerve sheath tumors. [11] In malignant tumors, complete resection is the only known curative treatment (with a sufficiently wide margin or even amputation to improve prognosis). [12]
A malignant peripheral nerve sheath tumor (MPNST) is a form of cancer of the connective tissue surrounding peripheral nerves. Given its origin and behavior it is classified as a sarcoma . About half the cases are diagnosed in people with neurofibromatosis ; the lifetime risk for an MPNST in patients with neurofibromatosis type 1 is 8–13%. [ 2 ]
A nerve sheath tumor is a type of tumor of the nervous system (nervous system neoplasm) which is made up primarily of the myelin surrounding nerves.Nerve sheath tumors can be benign or malignant, and may affect both the peripheral and central nervous systems.
Management of brachial or lumbosacral plexopathy depends on the underlying cause. No matter the cause of plexopathy, physical therapy and/or occupational therapy may promote recovery of strength and improve limb function. In the case of a mass lesion causing compression of the brachial or lumbosacral plexus, surgical decompression may be warranted.
Schwannomas: Tumors arising from Schwann cells, which produce the myelin sheath surrounding peripheral nerves. A common subtype is the vestibular schwannoma (acoustic neuroma). [5] Neurofibromas: Benign tumors that grow on nerves, commonly associated with neurofibromatosis type I (NF1). [2]
Wallerian degeneration is an active process of degeneration that results when a nerve fiber is cut or crushed and the part of the axon distal to the injury (which in most cases is farther from the neuron's cell body) degenerates. [1]
Malignant melanotic nerve sheath tumor (previously known as melanotic schwannoma) is a rare aggressive peripheral nerve sheath tumor that typically develops in conjunction with spinal or visceral autonomic nerves, consisting uniformly of Schwann cells displaying melanocytic differentiation.
Microsurgery can be used approaching the area from above the collar bone (supraclavicular) followed by neurolysis of the brachial plexus, removal of the scalene muscle (scalenectomy), and the release of the underlying (subclavicular) blood vessels. This approach avoids the use of resection, and has been found to be an effective treatment. [24]