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.
Radiosurgery involves the precise delivery of sharply collimated high-energy beams of radiation to a distinct target volume along selected trajectories. Historically, accurate targeting required the application of a stereotactic frame, thus limiting the use of this procedure to single treatments of selected intracranial lesions. However, the scope of radiosurgery has undergone a remarkable broadening since the introduction of image-guided robotic radiosurgery. Recent developments in real-time image guidance provide an effective frameless alternative to conventional radiosurgery and allow both the treatment of lesions outside the skull and the possibility of performing hypofractionation. As a consequence, targets in the spine, chest and abdomen can now also be radiosurgically ablated with submillimetric precision. Meanwhile, the combination of image guidance, robotic beam delivery, and non-isocentric inverse planning can greatly enhance the conformality and homogeneity of radiosurgery. The aim of this article is to describe the technological basis of image-guided radiosurgery and provide a perspective on future developments. The current clinical usage of robotic radiosurgery will be reviewed with an emphasis on those applications that may represent a major shift in the therapeutic paradigm.
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