Tumor growth rate and treatment response are crucial parameters for risk stratification and therapy monitoring of patients with NF2. The efficacy of this drug on NF2 associated VS has subsequently been confirmed in other studies 5, 6, 7, 8, 9.
In 2009 bevacizumab-given off-label for compassionate use-showed tumor reduction and hearing improvement in patients with NF2 4. VS are benign in nature but can cause severe neurological deficits and deafness as a result of local tumor growth 3. Hallmarks of NF2 are bilateral vestibular schwannomas (VS), peripheral schwannomas, cranial or spinal meningiomas and ependymomas 2. Neurofibromatosis type 2 (NF2) is a rare neurogenetic tumor predisposition disorder with an estimated incidence of about 1:33.000 live births 1. For precise volumes it is mandatory to use the same volumetric tools for all measurements. Volumetric assessment of MRI images is associated to an intrinsic risk of miscalculation. OsiriX showed the shortest analysis time.
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For the actual tumors, the estimated volumes with all three software tools showed a low coefficient of variability, but the mean volume estimates differed among the tools. The mean volume estimated by all three software programs differed significantly from the true volume of the phantoms, but OsiriX and BrainLab gave estimates that were not significantly different from each other. The software was compared with regard to accuracy and reproducibility of the measurements and time required for analysis. We performed volumetric measurements on phantoms with predefined volumes (0.1 to 8.0 ml) and tumors seen on 32 head MRI scans from eight NF2 patients with BrainLab, ITK-Snap, or OsiriX. We present a statistical and methodical comparison of three volumetric image analysis tools. Volumetric tumor measurements are essential to assess the efficacy of new therapies. Hallmarks are bilateral benign vestibular schwannomas, which can lead to deafness or brainstem compression. Neurofibromatosis type 2 is a neurogenetic disorder with an incidence of about 1:33.000.