Potential of HSP90 Inhibitors to Treat Neurofibromatosis-Related Tumors

摘要: Neurofibromatosis type 1 and 2 (NF1 NF2) are two distinct tumor predisposition syndromes in which affected patients develop benign or malignant tumors of the nervous system. NF1 is characterized by development neurofibromas, peripheral nerve sheath gliomas resulting from mutations gene, encoding neurofibromin. NF2 primarily schwannomas, meningiomas ependymomas merlin. Current therapeutic options limited to conservative monitoring, repeated surgical resection radiotherapy. Unfortunately these not optimal for treating recurring life-threatening genetic syndromes. Recent advances understanding molecular pathways involved tumorigenesis neurofibromatosis-related neoplasms robust mouse models has allowed identification promising drug targets chemotherapy, including HSP90. In an model, apoptosis induction cells with proteotoxic stress was achieved a combination mTOR HSP90 inhibitors. cell NF2, inhibitors have been used target multiple signaling activated. This chapter summarizes rationale recent developments use as potential therapies neurofibromatosis tumors.