Which one of the following is the most common tumor associated with type I neurofibromatosis?

NEET PG
#1

Which one of the following is the most
common tumor associated with type I
neurofibromatosis ?
a) Optic nerve glioma
b) Meningioma
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
present.

  1. 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.

  1. Axillary or inguinal freckling
  2. Two or more Lisch nodules.
    Lisch nodules are hamartomas located within the iris.
  3. 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.
  4. A distinctive osseous lesion.
    Sphenoid dysplasia or cortical thinning of long bones.
  5. Optic glioma
  6. 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.
    Short stature