Understanding the Role of Wnt Signaling in Schwann Cell Tumors

Abstract

Malignant Peripheral Nerve Sheath Tumors (MPNSTs) originate in the Schwann cell and often occur in patients with Neurofibromatosis Type 1 (NF1), but can also form spontaneously (3). NF1 is a genetic disease that occurs in 1 in 3,000 live births, and predisposes patients to benign neurofibromas. 10% of NF1 patients will have one of their benign neurofibromas undergo malignant transformation into an MPNST, the leading cause of death in NF1 patients (3). The average survival rate with patients with MPNSTs is 21 months and due to the lack of complete understanding of the genetic basis for MPNST development, the common treatments for these MPNSTs are surgery and non-specific chemotherapy (3). A current goal in the field is to understand which signaling pathways drive MPNST development and progression, with the hopes of discovering novel targeted therapies to improve the treatment these patients.