Safety of Radiosurgery Applied to Conditions with Abnormal Tumor Suppressor Genes
2007
eurofibromatosis-2 (NF2) and von Hippel-Lindau disease (VHL) are rare conditions that arise from abnormalities in tumor suppressor genes (7, 14). Individuals affected are predisposed to develop multiple nervous system tumors. Although the tumors themselves are nearly always histologically benign, their direct effects on neural structures and the morbidity that may be associated with surgery for these lesions can result in considerable disability. Furthermore, these patients face an additional burden: even when major surgery is undertaken to totally excise a tumor, they are not cured of the condition; their genetic tendency means that they are expected to develop further tumors with time. Reflecting these considerations, there has been debate regarding the role of surgery in NF2, with an increasing emphasis being placed on conservative management strategies, avoiding or
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