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Neurofibroma

Neurofibroma1

Neurofibroma2

General Information
Neurofibroma is a type of benign nerve sheath tumor, a class of tumor of the nervous system, which is comprised of myelin surrounding nerves. Neurofibromas may affect any nerve in the body but frequently grow on spinal nerve roots. The papules can appear as single lesions or multiple lesions. When presented with multiple lesions they are associated with Neurofibromatosis type I. Neurofibromatosis I is a genetic disorder also known as von Recklinghausen disease. These lesions present as soft pedunculated tan papules, which in the setting of neurofibromatosis can number in the hundreds. Currently there is no known treatment that can stop the progression of neurofibromas, the lesions can be surgically removed or the size can be reduced by radiation therapy.

Epidemiology
One lesion per 25,000 adults in the United States

Etiology
Unknown, NF1 gene predisposes patients to multiple neurofibromas

Pathogenesis
Neural tumor that can evolve from a papule to a nodule or polyp

Clinical
Soft pedunculated tan papules

Histology
Transformed Schwann cells with wavy contours and ovoid to elongated nuclei with fine dense heterochromatin

Bibliography
1. “Neurofibromatosis” (Online). February 2003. http://www.merck.com/mmhe/sec06/ch088/ch088d.html (visited: April 8, 2008)

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