LAUSR

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.