Pediatric Malignant Peripheral Nerve Sheath Tumor

  • Etiology: Neurofibroma that undergoes malignant transformation
  • Imaging MRI:
    — Large, infiltrative, often hemorrhagic soft tissue mass related to neurovascular bundle
    — Loss of target sign and irregular margins
    — T1WI: Internal bright signal
    — T1WI post contrast: Irregular enhancement
  • Imaging PET FDG:
    — Useful to detect malignant transformation of schwannoma with high SUV
    — The higher the maximum SUV the more likely that it is tumor
  • DDX:
  • Complications:
  • Treatment:
  • Clinical:
    — 25%-75% associated with neurofibromatosis type 1
    — 1-3% of patients with neurofibromatosis Type 1 develop malignant peripheral nerve sheath tumor

Radiology 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.

Clinical Cases of Malignant Peripheral Nerve Sheath Tumor

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.

Surgery Cases of Malignant Peripheral Nerve Sheath Tumor

Surgery image of malignant peripheral nerve sheath tumor
Surgical image through a right thoracotomy shows a large mass originating in the apex of the right chest. Normal lung can be seen in the upper right corner of the incision.