Malignant Peripheral Nerve Sheath Tumor

  • Etiology: neurofibroma that undergoes malignant transformation
  • Imaging: large, infiltrative, often hemorrhagic soft tissue mass related to neurovascular bundle
  • FDG PET: useful to detect malignant transformation of schwannoma with high SUV
  • Clinical: 25%-75% associated with neurofibromatosis type 1, 1-3% of patients with neurofibromatosis type 1 develop malignant peripheral nerve sheath tumor

Cases of Malignant Peripheral Nerve Sheath Tumor

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.