Part of the book: Biomaterials Science and Engineering
Part of the book: Biomaterials Science and Engineering
Part of the book: Regenerative Medicine and Tissue Engineering
Part of the book: Cells and Biomaterials in Regenerative Medicine
Neurofibromatosis (NF1) is a rare genetic disease that predisposes to tumors in the peripheral and central nervous systems and other neoplasms. Neurofibromas are complex benign tumors involving various cell types. Among them, plexiform neurofibromas are locally invasive peripheral nerve sheath tumors (PNs) that can cause disfigurement and functional limitations. Based on histopathological and magnetic resonance imaging (MRI) data, PNs display variable morphology and behavior. The appearance of distinct nodular lesions (DNLs) in PNs raises concerns about an increased risk of transformation into malignant peripheral nerve sheath tumors (MPNSTs). Surgery represents the primary treatment option for NF1-related PN, although recently, specific targeted agents, that is, MEK inhibitors, have been shown to be partially effective. Several surgical techniques have been proposed for PNs to decrease intraoperative bleeding and facilitate tumor excision of either diffuse PNs or rapidly growing nodular PN. However, despite improving surgical methods, complete tumor excision can be achieved in only a few cases. In this frame, it emerges evident that searching for appropriate surgical and pharmacological treatments for neurofibromas is a priority challenge. In this chapter, we will review current treatments approved by the scientific/clinical community, emphasizing the most recent progress in this field.
Part of the book: Neurofibromatosis