title: The biology and management of laryngeal neurofibroma.
abstract: OBJECTIVE
  To review the presentation of laryngeal neurofibroma, including its association with neurofibromatosis types 1 and 2, and present guidelines for its management.
  
  
  METHODS
  Retrospective study.
  
  
  METHODS
  Five pediatric patients with laryngeal neurofibroma, 4 girls (80%) and 1 boy (20%), were treated at a tertiary pediatric medical center from 1973 through 2003. Recorded data included age at initial presentation, sex, symptoms, significant medical and family history, preoperative evaluation, location of the tumor, surgical procedure, complications, outcome, and recurrence.
  
  
  RESULTS
  The 5 patients presented with stridor and cafe-au-lait spots at or shortly after birth. All patients were diagnosed as having neurofibromatosis type 1 by the established criteria. Studies evaluating the disease processes included plain radiography, computerized tomography, magnetic resonance imaging, barium swallow, and laryngoscopy and bronchoscopy under anesthesia. Pathologic examination of biopsy specimens from all patients showed neurofibromas with plexiform and/or diffuse features. Treatments included tracheotomy (n = 4), carbon dioxide laser excision (n = 4), modified neck dissection (n = 3), partial pharyngectomy (n = 1), supraglottic laryngectomy (n = 1), and endoscopic hemilaryngectomy (n = 1). Three patients were successfully decannulated. Follow-up ranged from 1 to 15 years. One patient was lost to follow-up. No evidence of malignant degeneration was noted.
  
  
  CONCLUSIONS
  Neurofibroma of the larynx is a rare condition that should be considered in the differential diagnosis of children with a submucosal laryngeal mass. In our series, all patients had associated neurofibromatosis type 1. Complete surgical excision is the treatment of choice in cases of localized small lesions. To prevent debilitating outcomes due to aggressive surgery, minimally invasive procedures (partial excision via endoscopic approach) may be preferable for larger lesions that infiltrate the surrounding vital structures. Long-term follow up of these patients is essential owing to the possibility of malignant transformation.
authors.affiliation: Department of Otolaryngology and Communication Disorders, Children's Hospital, Harvard Medical School, Boston, Mass, USA. reza.rahbar@childrens.harvard.edu
authors.forename: Reza
authors.initials: R
authors.lastname: Rahbar
authors.forename: Biana G
authors.initials: BG
authors.lastname: Litrovnik
authors.forename: Sara O
authors.initials: SO
authors.lastname: Vargas
authors.forename: Caroline D
authors.initials: CD
authors.lastname: Robson
authors.forename: Roger C
authors.initials: RC
authors.lastname: Nuss
authors.forename: Mira B
authors.initials: MB
authors.lastname: Irons
authors.forename: Trevor J
authors.initials: TJ
authors.lastname: McGill
authors.forename: Gerald B
authors.initials: GB
authors.lastname: Healy
mesh_terms: D000293:Adolescent; D001999:Bronchoscopy; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007822:Laryngeal Neoplasms / Q000175:diagnosis / Q000473:pathology / Q000628:therapy; D007825:Laryngectomy / Q000379:methods; D007828:Laryngoscopy; D053685:Laser Therapy; D008279:Magnetic Resonance Imaging; D008297:Male; D009455:Neurofibroma / Q000175:diagnosis / Q000473:pathology / Q000628:therapy; D009456:Neurofibromatosis 1 / Q000175:diagnosis / Q000473:pathology / Q000628:therapy; D010611:Pharyngectomy; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed; D014140:Tracheotomy