Pediatric Plexiform Neurofibroma

  • Etiology: heterogeneous mixture of Schwann cells / perineural cells / fibroblasts
  • Imaging: often involves nerve segments next to vertebral column, nodular tumor, multiple lesions along major nerves, individual nodules may break through outer nerve sheath and extend into surrounding soft tissues, often causes sphenoid wing erosion (dysplasia)
  • MRI: T2 hyperintense with +/- hypointense central focus (target sign), avid enhancement
  • Clinical: development begins early in childhood, almost exclusively seen in neurofibromatosis type 1 so is pathognomonic for neurofibromatosis type 1

Radiology 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.
CXR and MRI of neurofibromatosis type I
CXR shows a posterior mediastinal mass. Coronal and sagittal T1 MRI without contrast of the thoracic spine shows dural ectasia.

Clinical Cases of Plexiform Neurofibroma

Clinical image of neurofibromatosis type 1 with chest wall and arm plexiform neurofibromas
Clinical image shows extensive, large soft tissue masses involving the right arm and chest wall.