Neurofibromatosis type 1 with tracheobronchial neurofibromas: case report with emphasis on tomographic findings

J Thorac Imaging. 2008 Aug;23(3):194-6. doi: 10.1097/RTI.0b013e31816b6bc0.

Abstract

A 65-year-old man with neurofibromatosis type 1 (NF-1) presented with a 3-month history of dyspnea and cough. The chest spiral computed tomography scan showed multiple neurofibromas in the larynx and tracheobronchial tree. Bronchoscopy was performed and the biopsy confirmed the diagnosis of benign neurofibroma. The upper airway involvement in NF-1 is rare, and the diagnosis on the basis of the clinical manifestation may be difficult. The clinical manifestations of patients with tracheobronchial neurofibromas are mostly related to airway obstruction, presenting with cough, wheezing, and dyspnea. Our case illustrates the value of spiral computed tomography imaging in providing an excellent anatomic definition of this rare manifestation of NF.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Humans
  • Male
  • Neurofibromatosis 1 / diagnostic imaging*
  • Tomography, Spiral Computed*
  • Tracheal Neoplasms / diagnosis
  • Tracheal Neoplasms / diagnostic imaging*
  • Tracheal Neoplasms / pathology