This Article  • Full text  • PDF  • Reply to article  • Send to a friend  • Save in My Folder  • Save to citation manager  • Permissions  Citing Articles  • Contact me when this article is cited  Related Content  • Similar articles in this journal  Topic Collections  • Laryngology/ Speech/ Language Pathology  • Neoplasms of Head & Neck  • Pediatric Otolaryngology  • Alert me on articles by topic  Social Bookmarking   What's this?

Reza Rahbar, DMD, MD; Biana G. Litrovnik, MD; Sara O. Vargas, MD; Caroline D. Robson, MD; Roger C. Nuss, MD; Mira B. Irons, MD; Trevor J. McGill, MD; Gerald B. Healy, MD

Arch Otolaryngol Head Neck Surg. 2004;130:1400-1406.

Objectives  To review the presentation of laryngeal neurofibroma, including its association with neurofibromatosis types 1 and 2, and present guidelines for its management.

Design  Retrospective study.

Patients  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 café-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.

Author Affiliations: Departments of Otolaryngology and Communication Disorders (Drs Rahbar, Nuss, McGill, and Healy), Pathology (Dr Vargas), Radiology (Dr Robson), and Division of Genetics (Dr Irons), Children’s Hospital; Department of Otology and Laryngology (Drs Rahbar, Nuss, McGill, and Healy), Harvard Medical School (Drs Litrovnik, Vargas, Robson, and Irons), Boston, Mass. Dr Litovnik is now with the New York University Medical Center, New York University School of Medicine, New York.

CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    Twitter     What's this?