Medical expert of the article
New publications
Neurofibroma
Last reviewed: 07.07.2025
All iLive content is medically reviewed or fact checked to ensure as much factual accuracy as possible.
We have strict sourcing guidelines and only link to reputable media sites, academic research institutions and, whenever possible, medically peer reviewed studies. Note that the numbers in parentheses ([1], [2], etc.) are clickable links to these studies.
If you feel that any of our content is inaccurate, out-of-date, or otherwise questionable, please select it and press Ctrl + Enter.
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.
What do need to examine?