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Laryngeal involvement in neurofibromatosis
T O Willcox1, S I Rosenberg, S D Handler
1Department of Otorhinolaryngology: Head and Neck Surgery, University of Pennsylvania Medical Center, Philadelphia.
Ear, Nose, & Throat Journal
|December 1, 1993
Summary
Neurofibromas in the larynx are rare but important in diagnosing supraglottic masses. Surgical excision is preferred, but incomplete removal may be necessary to avoid debilitating surgery.
Area of Science:
- Otolaryngology
- Oncology
- Genetics
Background:
- Neurofibromas are uncommon manifestations of neurofibromatosis.
- Laryngeal neurofibromas present as submucosal supraglottic masses.
- Differential diagnosis for supraglottic masses should include neurofibromas.
Observation:
- Plexiform neurofibromas are poorly circumscribed and infiltrative, unlike non-plexiform types.
- Complete surgical excision is the primary treatment for laryngeal neurofibromas.
- Aggressive surgery may be debilitating, making incomplete excision a viable alternative.
Findings:
- Complete surgical excision is the treatment of choice for laryngeal neurofibromas.
- Incomplete excision may be preferable to avoid severe functional deficits.
- Plexiform neurofibromas exhibit infiltrative growth patterns.
- Tracheostomy may be required in some cases.
- Sarcomatous degeneration is a rare but serious complication with a poor prognosis.
Implications:
- Early consideration of neurofibromas in the differential diagnosis of supraglottic masses is crucial.
- Surgical management requires careful consideration of tumor type and potential for debilitating morbidity.
- Awareness of sarcomatous degeneration is important for prognosis and patient management.