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Neurol India. 2019 Jan-Feb;67(Supplement):S38-S44. doi: 10.4103/0028-3886.250697.

Peripheral nerve tumors in neurofibromatosis 1: An overview on management and indications for surgical treatment in our experience.

Author information

1
Brachial Plexus and Peripheral Nerve Surgery Unit, Neuro Spinal Hospital, Dubai, United Arab Emirates.

Abstract

Neurofibromatosis 1 (NF1) is associated with peripheral nerve tumors (PNTs) in about 30% of cases. In comparison with sporadic forms, NF1 PNTs present some peculiarities: (1) A large prevalence of neurofibromas; (2) the presence of pathognomonic tumoral forms (plexiform neurofibromas); and, (3) a higher incidence (lifetime risk is equal to 8-13%) and an earlier age of onset (2-3 versus 3-6 decades) of malignant peripheral nerve sheath tumors (MPNSTs). For fear of inducing neurological complications, surgical removal of PNTs is generally recommended for symptomatic tumors only. Yet, it can be safely performed by surgeons with expertise in the field. A valid preventive strategy is also essential. Based on the evidence that in NF1, one-third of MPNSTs are consequent to malignant transformation of pre-existing benign tumors, a more aggressive surgical attitude should be advocated.

KEYWORDS:

Malignant peripheral nerve sheath tumor; neurofibroma; neurofibromatosis; peripheral nerve tumor; plexiform neurofibroma; schwannoma

PMID:
30688231
DOI:
10.4103/0028-3886.250697
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