Neurofibroma

General Information
Probably the most common benign peripheral nerve tumor, neurofibroma is a
tumor of the nerve fiber.
On occasion a solitary neurofibroma occurs, but more frequently they occur as
part of neurofibromatosis. Neurofibromatosis is a genetic disorder that may be
associated with multiple neurofibromas. The disease is transmitted by an
autosomal dominant mutant gene.
There are two types of neurofibromatosis.
Type 1 (von Recklinghausen's disease) affects 1 in 3000 people; however, the
majority of cases are mild with a limited number of tumors.
Type 2, which is more severe, affects 1 in 50,000 people.
Symptoms
The spectrum of lesions in von Recklinghausen's disease varies widely. For
example, the neurofibromas may involve small skin nerves and appear as small
bumps, or they may be confined to large nerves and produce elephantiasis, or
enlargement of affected body parts.
Diagnosis
Neurofibromatosis 1:The diagnosis requires two or more of the following:
Six or more brownish discolorations on the skin, more than 5mm in greatest
diameter in pre-adolescents and greater than 15 mm in adolescents and adults.
Two or more neurofibromas.
Freckling in the arm pits or groin regions.
Optic glioma.
Two or more Lisch nodules, or small masses on the iris of the eyes.
Bone lesions, called sphenoid dysplasia.
A first degree relative with neurofibromatosis type 1.
Neurofibromatosis 2: The diagnosis requires either of the following:
Eighth cranial nerve masses on both sides (bilateral).
An immediate relative with neurofibromatosis 2, and either an eighth nerve mass
on one side or two of the following: neurofibroma, meningioma, glioma
or schwannoma.
Treatment
The management of neurofibromas depends upon the symptoms. They do not
typically need to be surgically removed.
Surgical exploration and excision may be required under the following
circumstances: type of diagnosis, pain, cosmetic considerations, progressive
neurological complications, compression of adjacent tissues and suspicion of a
malignant tumor.
Painful neurofibromas occur just under the skin. These globular lesions may
occur in the hand or other surfaces that are subject to pressure. Typically these
lesions involve small bundles of superficial nerves and can be removed without
creating a meaningful neurological deficit.
The Neuro-ICU cares for patients with all types of neurosurgical and neurological

injuries, including stroke, brain hemorrhage, trauma and tumors. We work in

close cooperation with your surgeon or medical doctor with whom you have had
initial contact. Together with the surgeon or medical doctor, the NeuroICU
attending physician and team members direct your family member's care while in
the ICU. The NeuroICU team consists of the bedside nurses, nurse practitioners,
physicians in specialty training (Fellows) and attending physicians.