Adult Supraglottic Rhabdomyoma with Extralaryngeal Extension: Surgical Excision and Reconstruction with Aortic Homograft

Abstract

Objectives: 1) Review the presentation, pathology, and treatment of rhabdomyomas. 2) Recognize the potential need for open resection with reconstruction as a treatment of benign laryngeal lesions in rare cases. Methods: A 67-year-old female presented with dyspnea and hoarseness. On examination she had a soft, mobile, centrally-located neck mass. Fiberoptic laryngoscopy revealed a large, submucosal supraglottic mass. Computed tomography (CT) showed a 3-cm supraglottic tumor with extralaryngeal extension. Needle aspiration suggested rhabdomyoma, confirmed by direct laryngoscopy with biopsy. She subsequently underwent awake tracheostomy, hemilaryngectomy, and reconstruction with aortic homograft. Pathology showed adult rhabdomyoma with representative large, polygonal cells with abundant cytoplasm, focal cross-striations, and occasional “strap” cells. Three weeks postoperatively she was decannulated and found to have a functional voice and swallow. After six months of follow-up she is without evidence of recurrence. Conclusions: Laryngeal rhabdomyoma is a rare benign clinical entity, which may present with airway distress and extralaryngeal extent. Treatment is complete surgical excision, possibly requiring open surgery with reconstruction. Aortic homograft reconstruction is safe, is technically straight-forward, and has good postoperative results.