What is the malignancy risk of neurofibromas?

Updated: Jan 30, 2020
  • Author: Ginard I Henry, MD; Chief Editor: Gregory Gary Caputy, MD, PhD, FICS  more...
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Answer

Answer

Individuals with NF-1 harbor a 7–13% lifetime risk of developing a malignant peripheral nerve sheath tumor, which usually arises in preexisting plexiform or focal subcutaneous neurofibromas. Using the normal clinical signs to evaluate for a potential malignant or transforming premalignant lesion is challenging. In patients with NF-1, new lesions develop and grow continually, so close surveillance is recommended. Neurofibroma can occur in major peripheral nerves, which can cause disruption of nerve function. [24] Neurofibromas commonly occur within the substance of the nerve, which creates a severe challenge to remove the tumor without causing further nerve dysfunction. Internal neurolysis and nerve grafting are performed as indicated for neurofibromas resection.


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