Amyloidosis of the larynx.

Amyloidosis is characterized by the accumulation of insoluble fibrillar protein (amyloid) in tissues and organs throughout the body. Some deposition processes are local and some are systemic. Of the several types of amyloidosis, nodular amyloidosis is the type found most frequently in the larynx and nasopharynx, as well as in the trachea and lungs.

A 48-year-old basketball coach presented with a 2.5-year history of progressive hoarseness, vocal fatigue, loss of high and low range, and pain when speaking. His problems had become severe enough to force him to retire from coaching. Prior to his visit, his otolaryngologist had diagnosed amyloidosis of the larynx.

Histopathologic evaluation on H&E staining of a soft-tissue mass revealed normal surface squamous epithelium, chronic subepithelial inflammatory infiltrate, and diffuse eosinophilic intercellular deposits (some of which caused pressure necrosis of the surrounding stoma) (figure 1). Sections stained with Congo red dye demonstrated the characteristic yellow and apple-green birefringence, and the lesion had the beta-pleated sheet structure and antiparallel conformation by low-angle x-ray diffraction, typical of amyloidosis. (figure 2).

The patient was referred to the senior author (R.T.S.) for further treatment. A thorough systemic workup revealed no evidence of amyloid outside the head and neck. Physical examination and videostroboscopy detected a large left supraglottic mass that obscured visualization of the anterior two-thirds of the left vocal fold (figure 3A). Endoscopy also revealed a partial supraglottic obstruction of the airway and a good subglottic airway, despite evidence of a tracheal mass (figure 3B). Videostroboscopy also detected both a pharyngeal (figure 3C) and a tongue base (figure 3D) mass. Surgical resection of the supraglottic mass resulted in an improved voice.

From the Department of Otolaryngology-Head and Neck Surgery, Thomas Jefferson University, Philadelphia (Dr. Sataloff and Dr. M. Abaza); the Department of Otolaryngology-Head and Neck Surgery, Graduate Hospital, Philadelphia (Dr. Sataloff); the Department of Surgery, MCP-Hahnemann School of Medicine, Philadelphia (Dr. N. Abaza); and the American Institute for Voice and Ear Research, Philadelphia (Ms. Markiewicz and Ms. Hawkshaw).