Neurofibromas

is a lump (tumor) that contains nerve

tissue and is benign
There are two main forms of neurofibroma :
1. Type 1 (NF1, von Recklinghausen's disease)
2. Type 2 (NF-2, formerly known as bilateral
acoustic NF or neurofibromacentral)
 epidemiology
• Type 1 NF: 85% of all NF
• NF type 1: 1 in 5000 births
• 30-50% of cases of type 1 NF have no family
history
• Type 2 NF: 1 out of 210,000 births
 Etiology
• NF type 1: by mutations of genes located on
chromosome 17
• NF type 2: by mutations of genes located on
chromosome 22
 Clinical manifestations
• NF type 1
1. There are 6 or more café-au-lait-spots with
the largest diameter> 5mm in prepuberty,>
15mm in post puberty
2. Some NF (tumors on or below or hanging
skin)
3. Freckling
4. Lisch nodules
5. Optic glioma
• NF type 2
• Marked: several tumors and lesions in the
brain and spinal cord
 Diagnosis
• Use the National Institute of Health (NIH)
criteria
• NF type 1 must meet 2 or more criteria
• NF type 2 must meet one of 2 criteria
 treatment
• There is no cure for NF type 1 or 2
• Surgery to remove the tumors is the only
treatment
• Depending on location of the tumom and
treating it in a timely matter before the
growth becomes to large and inoperable
• If (NF) is found in childhood, the child should
be seen every 6 months to be monitored
 Prognosis
• If there are no complications, the life
expectancy of patients with neurofibromatosis
will be good. With the right education, people
with NF can live a normal life.