Plexiform Neurofibroma

  • Etiology: heterogeneous mixture of Schwann cells / perineural cells / fibroblasts
  • Imaging: often causes sphenoid wing erosion (dysplasia)
  • Clinical: almost exclusively seen in neurofibromatosis type 1

Cases of Plexiform Neurofibroma

CXR and CT of plexiform neurofibroma in neurofibromatois type 1
CXR PA shows a large soft tissue mass in the apex of the right lung which causes thinning of the right 1st-5th ribs posteriorly. CT without contrast of the chest shows multiple small nodules throughout the soft tissues of the chest wall.
CXR and CT of malignant peripheral nerve sheath tumor in neurofibromatosis type 1
CXR PA and lateral shows a right apical thoracic mass with associated rib thinning that had rapidly increased in size in a short interval. Axial CT without contrast of the chest better defined the right apical mass and demonstrated multiple serpiginous nodules in the soft tissues of the right anterior chest wall.