Neurofibromatosis type 1 with tracheobronchial neurofibromas: case report with emphasis on tomographic findings.
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.
Irion
Klaus Loureiro
KL
Department of Radiology, Cardiothoracic Center, Liverpool NHS Trust, UK.
Gasparetto
Taisa Davaus
TD
Marchiori
Edson
E
Hochhegger
Bruno
B
Rubin
Adalberto Sperb
AS
D000368:Aged; D006801:Humans; D008297:Male; D009456:Neurofibromatosis 1 / Q000000981:diagnostic imaging*; D036542:Tomography, Spiral Computed; D014134:Tracheal Neoplasms / Q000175:diagnosis / Q000000981:diagnostic imaging* / Q000473:pathology