Model systems for neurofibroma and malignant peripheral nerve sheath tumor

Although clinically benign, neurofibromas that form in type 1 neurofibromatosis (NF1) require surgical removal when disfiguring or causing morbidity. Surgery is not always feasible, and no successful molecular therapy has been identified. Plexiform neurofibromas associated with large deep nerves show increased risk for transformation to life threatening malignant peripheral nerve sheath tumor (MPNST). In vitro, in vivo and in silico models of neurofibromas and MPNST are being used to develop a preliminary molecular model of peripheral nerve tumor progression in NF1. Although each neurofibroma or MPNST model has strengths and limitations, together the models provide a platform to analyze tumor pathogenesis, and guide drug discovery for NF1.

Section editors:Nikolina Vlatovic – University of Liverpool, Liverpool, UKMark T. Boyd – University of Liverpool, Liverpool, UK