Consensus recommendations for current treatments and accelerating clinical trials for patients with neurofibromatosis type 2.
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Consensus recommendations for current treatments and accelerating clinical trials for patients with neurofibromatosis type 2. / Blakeley, Jaishri O; Evans, D Gareth; Adler, John; Brackmann, Derald; Chen, Ruihong; Ferner, Rosalie E; Hanemann, C Oliver; Harris, Gordon; Huson, Susan M; Jacob, Abraham; Kalamarides, Michel; Karajannis, Matthias A; Korf, Bruce R; Mautner, Viktor Felix; McClatchey, Andrea I; Miao, Harry; Plotkin, Scott R; Slattery, William; Stemmer-Rachamimov, Anat O; Welling, D Bradley; Wen, Patrick Y; Widemann, Brigitte; Hunter-Schaedle, Kim; Giovannini, Marco.
In: AM J MED GENET A, Vol. 158A, No. 1, 1, 2012, p. 24-41.Research output: SCORING: Contribution to journal › SCORING: Journal article › Research › peer-review
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TY - JOUR
T1 - Consensus recommendations for current treatments and accelerating clinical trials for patients with neurofibromatosis type 2.
AU - Blakeley, Jaishri O
AU - Evans, D Gareth
AU - Adler, John
AU - Brackmann, Derald
AU - Chen, Ruihong
AU - Ferner, Rosalie E
AU - Hanemann, C Oliver
AU - Harris, Gordon
AU - Huson, Susan M
AU - Jacob, Abraham
AU - Kalamarides, Michel
AU - Karajannis, Matthias A
AU - Korf, Bruce R
AU - Mautner, Viktor Felix
AU - McClatchey, Andrea I
AU - Miao, Harry
AU - Plotkin, Scott R
AU - Slattery, William
AU - Stemmer-Rachamimov, Anat O
AU - Welling, D Bradley
AU - Wen, Patrick Y
AU - Widemann, Brigitte
AU - Hunter-Schaedle, Kim
AU - Giovannini, Marco
PY - 2012
Y1 - 2012
N2 - Neurofibromatosis type 2 (NF2) is a tumor suppressor syndrome characterized by bilateral vestibular schwannomas (VS) which often result in deafness despite aggressive management. Meningiomas, ependymomas, and other cranial nerve and peripheral schwannomas are also commonly found in NF2 and collectively lead to major neurologic morbidity and mortality. Traditionally, the overall survival rate in patients with NF2 is estimated to be 38% at 20 years from diagnosis. Hence, there is a desperate need for new, effective therapies. Recent progress in understanding the molecular basis of NF2 related tumors has aided in the identification of potential therapeutic targets and emerging clinical therapies. In June 2010, representatives of the international NF2 research and clinical community convened under the leadership of Drs. D. Gareth Evans (University of Manchester) and Marco Giovannini (House Research Institute) to review the state of NF2 treatment and clinical trials. This manuscript summarizes the expert opinions about current treatments for NF2 associated tumors and recommendations for advancing therapies emerging from that meeting. The development of effective therapies for NF2 associated tumors has the potential for significant clinical advancement not only for patients with NF2 but for thousands of neuro-oncology patients afflicted with these tumors.
AB - Neurofibromatosis type 2 (NF2) is a tumor suppressor syndrome characterized by bilateral vestibular schwannomas (VS) which often result in deafness despite aggressive management. Meningiomas, ependymomas, and other cranial nerve and peripheral schwannomas are also commonly found in NF2 and collectively lead to major neurologic morbidity and mortality. Traditionally, the overall survival rate in patients with NF2 is estimated to be 38% at 20 years from diagnosis. Hence, there is a desperate need for new, effective therapies. Recent progress in understanding the molecular basis of NF2 related tumors has aided in the identification of potential therapeutic targets and emerging clinical therapies. In June 2010, representatives of the international NF2 research and clinical community convened under the leadership of Drs. D. Gareth Evans (University of Manchester) and Marco Giovannini (House Research Institute) to review the state of NF2 treatment and clinical trials. This manuscript summarizes the expert opinions about current treatments for NF2 associated tumors and recommendations for advancing therapies emerging from that meeting. The development of effective therapies for NF2 associated tumors has the potential for significant clinical advancement not only for patients with NF2 but for thousands of neuro-oncology patients afflicted with these tumors.
KW - Humans
KW - Research Design
KW - Clinical Trials as Topic
KW - Consensus
KW - Endpoint Determination
KW - Standard of Care
KW - Meningioma/diagnosis/therapy
KW - Neurofibromatosis 2/diagnosis/genetics/therapy
KW - Radiosurgery
KW - Humans
KW - Research Design
KW - Clinical Trials as Topic
KW - Consensus
KW - Endpoint Determination
KW - Standard of Care
KW - Meningioma/diagnosis/therapy
KW - Neurofibromatosis 2/diagnosis/genetics/therapy
KW - Radiosurgery
M3 - SCORING: Journal article
VL - 158A
SP - 24
EP - 41
JO - AM J MED GENET A
JF - AM J MED GENET A
SN - 1552-4825
IS - 1
M1 - 1
ER -
