Tumor Suppressor Mutations and Growth Factor Signaling in the Pathogenesis of NF1-Associated Peripheral Nerve Sheath Tumors

Abstract excerpt

Patients with neurofibromatosis type 1 (NF1), one of the most common genetic disease affecting the nervous system, develop multiple neurofibromas that can transform into aggressive sarcomas known as malignant peripheral nerve sheath tumors (MPNSTs). Studies of human tumors and newly developed transgenic mouse models indicate that Schwann cells are the primary neoplastic cell type in neurofibromas and MPNSTs and...

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