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Neurofibroma

 
, medical expert
Last reviewed: 07.07.2025
 
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Plexiform neurofibroma

Plexiform (diffuse) neurofibroma is the most common tumor of the peripheral nerves of the orbit and occurs almost exclusively in association with neurofibromatosis type I.

Plexiform (diffuse) neurofibroma appears in young children as a periorbital swelling.

Symptoms of neurofibroma

  • Diffuse orbital lesion with disfiguring hypertrophy of tissues around the eye.
  • The eyelid lesion causes mechanical ptosis of a characteristic S-shaped configuration. Palpation of the altered tissues resembles a "bag of worms".
  • Pulsation of the eye without accompanying noise (better detected with applanation tonometry) may occur when neurofibroma is combined with a congenital defect of the greater wing of the sphenoid bone.

Treatment is extremely difficult. Surgery is not indicated, if at all possible, because of the complex relationship between the tumor and important orbital structures.

Isolated neurofibroma

Isolated (localized) neurofibroma is less common and is associated with neurofibromatosis type I in approximately 10% of cases.

Isolated (localized) neurofibroma manifests itself in the 3rd or 4th decade of life as mild, moderately painful exophthalmos combined with decreased vision or limited eye mobility.

Surgical treatment has a direct indication, since the tumor is well delineated and relatively poor in vessels.

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