Tumors of the peripheral nerves and plexuses.
Peripheral nerve tumors are a diverse group of lesions histologically and in their clinical behavior. The genetic disorders neurofibromatosis type 1 and 2 and schwannomatosis are significant risk factors for the development of peripheral nerve tumors. An understanding of these disorders is important in allowing appropriate management. Active treatment of peripheral nerve tumors is reserved for lesions that are malignant or causing neurologic dysfunction, pain, compressive symptomatology, or cosmetic concern. The mainstay of treatment is surgical intervention, the nature of which will vary with the type of tumor and anatomical location. In the case of malignant tumors, adjuvant chemotherapy and radiotherapy are commonly used. Developments in the understanding of the genetics and molecular biology of peripheral nerve tumors are opening up potentially exciting new avenues of treatment. The prognosis of benign peripheral nerve tumors is excellent, with a recurrence rate of just 5% or less after successful surgery. Malignant tumors have proven to be more challenging, with a much higher recurrence rate and a 5-year survival rate of 64%.
Huang
Jason H
JH
Department of Neurosurgery, Hospital of the University of Pennsylvania, 3400 Spruce Street, Silverstein 3, Philadelphia, PA 19104, USA. huangj@uphs.upenn.edu
Johnson
Victoria E
VE
Zager
Eric L
EL