The neurofibromatoses: hereditary predisposition to multiple peripheral nerve tumors

Neurosurgeons must be aware of the neurofibromatoses because they frequently present with nervous system tumors. Such patients develop numerous peripheral nerve sheath tumors; fortunately, only a few of these tumors are symptomatic or malignant. The tumor burden in individual patients can be remarkable, however, producing significant morbidity and requiring extensive or repeated care by a skilled neurosurgeon. Because it is impossible to predict which of the deep neurofibromas will undergo malignant transformation, it behooves clinicians and patients to be aware of any change in symptoms or growth of tumors, because early resection of MPNST offers the only chance for cure.