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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]
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.
Immunoglobulin and steroids are the first line choices for treatment. [citation needed] In severe cases of CIDP, when second-line immunomodulatory drugs are not efficient, autologous hematopoietic stem cell transplantation (HSCT) is sometimes performed. The treatment may induce long-term remission even in severe treatment-refractory cases of CIDP.
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.
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]
Brachial plexopathy is often caused from local trauma to the brachial plexus, as can happen from a dislocated shoulder.The disorder can also be secondary to compression or stretching of the brachial plexus (for example, during a baby's transit through the birth canal, in which case it may be referred to as Erb's Palsy or Klumpke's palsy). [2]
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]