Tumor suppressor mutations and growth factor signaling in the pathogenesis of NF1-associated peripheral nerve sheath tumors. I. The role of tumor suppressor mutations.

Abstract excerpt

Patients with neurofibromatosis type 1 (NF1), a common autosomal dominant tumor predisposition syndrome, develop benign cutaneous, intraneural, and plexiform neurofibromas and malignant peripheral nerve sheath tumors (MPNSTs), an aggressive form of Schwann cell neoplasm that frequently arises from plexiform neurofibromas. Impressive advances have been made in defining the molecular mechanisms responsible for...

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