Developing a better way to monitor the size of slow growing, complex tumors
There is currently no effective therapy for patients with peripheral nerve sheath tumors called plexiform neurofibromas resulting from neurofibromatosis type I (NF1), and surgery is only an option for a subset of patients with the disorder. Before 2004, there was not an accurate way to measure the growth of these tumors to track disease progression.
IRP researcher Brigitte Widemann, M.D., and colleagues developed a method called semi-automated volumetric MRI analysis to measure the tumors. The technique allows researchers to reproducibly and sensitively measure changes in tumor size and accurately define the time to disease progression or shrinkage as primary endpoints in clinical trials.
As a result of this study, semi-automated volumetric MRI analysis is now used nationwide to determine response in most clinical trials of therapies against neurofibromatosis type I and in preclinical trials of targeted agents for neurofibromas in animal models.
Solomon J, Warren K, Dombi E, Patronas N, Widemann B. (2004). Automated detection and volume measurement of plexiform neurofibromas in neurofibromatosis 1 using magnetic resonance imaging. Comput. Med. Imaging Graph. 28(5), 257-65. doi:10.1016/j.compmedimag.2004.03.002
Dombi E, Solomon J, Gillespie AJ, Fox E, Balis FM, Patronas N, Korf BR, Babovic-Vuksanovic D, Packer RJ, Belasco J, Goldman S, Jakacki R, Kieran M, Steinberg SM, Widemann, BC. (2007) NF1 plexiform neurofibroma growth rate by volumetric MRI: relationship to age and body weight. Neurology. 68(9), 643-7.
Wu J, Dombi E, Jousma E, Dunn RS, Kim MO, Kim A, Widemann BC, Cripe TP, Ratner N. (2012). Preclincial testing of sorafenib and RAD001 in the Nf(flox/flox); DhhCre mouse model of plexiform neurofibroma using magnetic resonance imaging. Pediatr. Blood Cancer. 58(2), 173-80. doi: 10.1002/pbc.23015