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Common presentation: focal, unilateral lesion affecting major peripheral nerves (sciatic, median, radial, brachial plexus) and their branches. Uncommon locations: cranial nerves, lateral ventricle, oral cavity, skin, and mandible. Bilateral or unilateral multifocal lesions are rare; Hybrid nerve sheath tumor Benign Very rare
Malignant peripheral nerve sheath tumors are a rare type of cancer that arise from the soft tissue that surrounds nerves. They are a type of sarcoma. Most malignant peripheral nerve sheath tumors arise from the nerve plexuses that distribute nerves into the limbs—the brachial and lumbar plexuses—or from nerves as they arise from the trunk. [5]
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 .
The brachial plexus is a network of nerves (nerve plexus) formed by the anterior rami of the lower four cervical nerves and first thoracic nerve (C5, C6, C7, C8, and T1).This plexus extends from the spinal cord, through the cervicoaxillary canal in the neck, over the first rib, and into the armpit, it supplies afferent and efferent nerve fibers to the chest, shoulder, arm, forearm, and hand.
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.
In progressive cases, the brachial plexus is also affected, causing pain and weakness in the muscles of the arm and hand with a symptomatology typical of thoracic outlet syndrome. The tumor can also compress the recurrent laryngeal nerve and from this a hoarse voice and "bovine" (non-explosive) cough may occur. [11] [5]
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]