Rare presentation of laryngeal neuroma in a patient with multiple endocrine neoplasia type two B

Abstract

AbstractObjective:We present a rare case of laryngeal mucosal neuroma, and its management, in a patient with multiple endocrine neoplasia type 2 B.Method:We present a case report and a review of the world literature concerning laryngeal mucosal neuroma and its association with multiple endocrine neoplasia type 2 B.Case report:An 11-year-old girl was diagnosed with multiple endocrine neoplasia type 2 B on the basis of clinical presentation and genetic testing. Several years later, whilst undergoing general anaesthesia for routine dental treatment, the anaesthetic team were concerned by masses on the patient's vocal folds, which they felt could compromise her airway. The ENT team were informed of the situation and an urgent microlaryngoscopy was performed. Biopsies taken at the time were reported as showing benign mucosal neuromas. A few months later, the patient presented with acute stridor, and flexible nasal endoscopy revealed recurrence. This episode settled without surgery.Conclusion:Multiple endocrine neoplasia syndromes are inherited autosomal cancer syndromes. Laryngeal mucosal neuroma can manifest as potentially airway compromising lesions in patients with multiple endocrine neoplasia type 2 B. Although apparently rare, we feel this case highlights the need for vigilance and prompt recognition of this treatable condition.