Tumors of the peripheral nervous system

Area of Science:

• Oncology
• Neurosurgery
• Genetics

Background:

• Schwannomas and neurilemomas are tumors arising from Schwann cells, the supporting cells of peripheral nerves.
• These tumors can be benign or malignant, with malignant forms requiring aggressive treatment.
• Neurofibromatosis (von Recklinghausen's disease) is a genetic disorder associated with an increased risk of these tumors.

Purpose of the Study:

• To review the treatment and outcomes of benign and malignant schwannomas and neurilemomas.
• To evaluate the efficacy of surgical extirpation and radiation therapy.
• To assess the impact of neurofibromatosis on prognosis.

Main Methods:

• Review of surgical and radiation therapy outcomes for patients with schwannomas and neurilemomas.
• Analysis of 100 patients with malignant neurilemomas, including those with and without von Recklinghausen's disease.
• Calculation of 10-year survival and "cure" rates.

Main Results:

• Surgical extirpation is the primary curative treatment for benign and malignant schwannomas.
• Radiation therapy provides palliation and prolongs life but does not cure malignant schwannomas.
• The 10-year "cure" rate for malignant neurilemomas was 32%, with similar survival rates for patients with and without von Recklinghausen's disease (30% vs. 39%).

Conclusions:

• Malignant schwannomas require radical surgical resection.
• While radiation offers palliation, chemotherapy and immunotherapy have limited roles in current treatment.
• Patients with neurofibromatosis have a significant risk of developing malignant neurilemomas, but survival rates are comparable to those with solitary tumors.