Plexiform neurofibromatosis of the mediastinum: CT appearance

Abstract

We studied the CT findings in four patients with pathologically proved thoracic plexiform neurofibromatosis. In all four patients, CT showed an infiltrative process and masses that involved the mediastinum along the distribution of the sympathetic chains, phrenic, and vagus nerves. The lesions had lower attenuation values than did chest-wall muscle. In one patient, CT revealed calcifications and peripheral enhancement of nodular components after IV infusion of contrast material. In the appropriate clinical setting, CT detection of lesions in the distribution of the mediastinal nerves strongly favors the diagnosis of plexiform neurofibromatosis.