Plexiform Neurofibromatosis of the Mediastinum: CT Findings

ABSTRACT

PURPOSE: To evaluate the findings and the role of CT in plexiform neuro-fibromatosis of the mediastinum. MATERIALS AND METHODS: We retropectively reviewed the CT scans of five patients with plexiform neurofibromatosis of the mediastinum. The CT scans were reviewed with attention to the distribution of the lesions, appearance and attenuation of mediastinal lesions, enhancement pattern after intravenous contrast infusion and associated findingssuch as intercostal neurofibroma. RESULTS: In all five patients CT scans demonstrated fusiform low attenuated masses which were oriented longitudinally and extended over multiple contiguous scans along the distribution of major mediastinal nerves. In four patients, mediastinal lesions appeared infiltrative, obliterating adjacent mediastinal fat plane. One patient had well defined fusiform masses along the major mediastinal nerves. Postcontrast enhanced CT scans revealed slight central enhancement in two patient and no contrast enhancement in three patients. Associated findings such as neurofibromas of intercostal nerves and sympathetic trunk, or subcutaneous neurofibromas were detected on CTscans in all five patients. CONCLUSION: Characteristic CT findings of low attenuation masses along the major mediastinal nerves are helpful to differentiate plexiform neurofibromatosis from mediastinal lymphadenopathy and to prevent from misreading as a malignant disease.