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Neurofibroma
Last reviewed: 19.11.2021
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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 combination with Type I neurofibromatosis.
Plexiform (diffuse) neurofibroma is manifested in children of early age and as a periorbital swelling.
Symptoms of Neurofibroma
- Diffuse lesion of the orbit with disfiguring tissue hypertrophy around the eye.
- The defeat of the eyelids causes mechanical ptosis of a characteristic S-shaped configuration. Palpator-modified tissues resemble a "packet with worms."
- The pulsation of the eye without accompanying noise (it is better to be detected with applanation tonometry) can be when a combination of neurofibroma with a congenital defect of the large wing of the base bone is detected.
Treatment is extremely difficult. Surgery is not indicated, if at all possible, due to the complex relationship between the tumor and the important structures of the orbit.
Isolated neurofibroma
Isolated (localized) neurofibroma is less common and is combined with type I neurofibromatosis in about 10% of cases.
Isolated (localized) neurofibroma manifests itself in 3 or 4 decades of life with an undefined moderately painful exophthalmos in combination with a decreased vision or limited mobility of the eye.
The treatment is surgical, it has a direct indication, since the tumor is well-delimited and relatively poor in blood vessels.