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Longitudinal Study of Neurofibromatosis 1 Associated Plexiform Neurofibromas
  1. Tracy Tucker (tbtucker{at}interchange.ubc.ca)
  1. University of British Columbia, Canada
    1. Jan M. Friedman (frid{at}interchange.ubc.ca)
    1. University of British Columbia, Canada
      1. Reinhard E Friedrich (rfriedri{at}uke.uni-hamburg.de)
      1. University Hospital Eppendorf, Germany
        1. Ralph Wenzel (ralphwenzel{at}freenet.de)
        1. MRI Institute Hamburg Othmarschen, Germany
          1. Carsten Fünsterer (mrpraxis{at}mrt-hamburg.de)
          1. MRI Institute Hamburg Othmarschen, Germany
            1. Victor-F Mautner (vmautner{at}uke.uni-hamburg.de)
            1. University Hospital Eppendorf, Germany

              Abstract

              Background: Plexiform neurofibromas occur in more than half of people with neurofibromatosis 1 (NF1). These benign tumours can be disfiguring and can cause complications such as bone erosion or respiratory or gastrointestinal tract obstruction. Plexiform neurofibromas can also progress to malignant peripheral nerve sheath tumours (MPNSTs), one of the leading causes of death among NF1 patients. Plexiform neurofibromas are clinically heterogeneous, and knowledge of their natural history is limited. In order to characterize the growth of plexiform neurofibromas better, we performed serial magnetic resonance imaging (MRI) in NF1 patients with such tumours.

              Methods: MRI was done on 44 plexiform neurofibromas in 34 NF1 patients (median age = 10 years; range 1-47 years). Each tumour was measured in two dimensions from the MRI scan, and the area and growth rate were calculated. The median length of follow-up was 6 years, with an average interval of 3 years between scans.

              Results: Thirty-six tumours remained stable in size throughout the period of follow-up. Eight tumours increased in size; all occurred in patients who were under 20 years of age when first studied. The single exception was a 55 year-old man who developed rapid tumour growth and pain in a plexiform neurofibroma that had been followed for 10 years. Biopsy showed the presence of a MPNST.

              Conclusion: Longitudinal MRI is a valuable means of monitoring the growth of plexiform neurofibromas in individuals with NF1.

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