Which one of the following is the most
common tumor associated with type I
a) Optic nerve glioma
c) Acoustic schwannoma
d) Low grade astrocytoma
Correct Answer - A
Ans. is ‘a’ i.e., Optic nerve glioma
Neurofibromatosis type I (Von-Recklinghewsen disease)
o NF-1 is diagnosed when any two of the following seven signs are
- Six or more cafe-au-lait macules
5 mm in prepupertal individuals
15 mm in postpubertal individuals
Cafe-au-fait spots are the hallmark of neurofibromatosis
and are present in almost 100% of the patient.
- Axillary or inguinal freckling
- Two or more Lisch nodules.
Lisch nodules are hamartomas located within the iris.
- Two or more neurofibroma or one plexiform neurofibroma.
Typically involve the skin, but may be situated along
peripheral nerves and blood vessels.
They are small, rubbery lesions with a slight purplish
discoloration of the overlying skin.
- A distinctive osseous lesion.
Sphenoid dysplasia or cortical thinning of long bones.
- Optic glioma
- A first degree relative with NF-1
Other findings are : -
Pseudoarthrosis of tibia.
Scoliosis is the most common orthopaedic problem in NF-1,
but is not specific enough to be included as a diagnostic criterian.