Neurofibroma

Epidemiology

Neurofibromas can arise from cranial as well as spinal nerves. These tumors may occur in isolation or as part of syndromes such as neurofibromatosis. Only 10% of patients with neurofibromas have neurofibromatosis. Neurofibromas usually present in the fourth or fifth decade of life. Neurofibroma in the neck typically involves the vagus nerve but may also arise from other cranial nerves and the sympathetic chain.

Clinical Findings

The usual presentation is that of a painless neck mass. However, associated cranial nerve symptoms may be evident such as paresthesia or cranial nerve palsy.

Pathology

Neurofibromas, in contrast to schwannomas, are nonencapsulated tumors. They contain all elements of the nerve of origin. Histology shows a mixture of Schwann’s cells and fibro-blasts dispersed among nerve fibers resulting in a fusiform dilation of the involved nerve. The matrix consists of mucopolysaccharides and an abundance of tissue fluid. The risk of malignant transformation into a neurofibrosarcoma is < 5%.

Treatment

Neurofibromas are treated by surgical excision. As the fusiform tumor arises from the nerve itself, excision of the tumor is followed by nerve repair.