Surgical rehabilitation for primary laryngeal amyloidosis.

A 35-year-old woman presented with near-complete aphonia. She had undergone excision of a laryngeal polyp 1 year earlier. Pathologic examination revealed that the lesion was consistent with laryngeal amyloidosis. Postoperatively, she had experienced an initial improvement in her voice, but over the following 10 months, the dysphonia had returned. She also reported dyspnea while supine.

Fiberoptic examination identified a polypoid mass that involved the left false vocal fold and ventricle (figure 1). Magnetic resonance imaging (MRI) demonstrated a homogenously enhancing submucosal mass in the left supraglottis that extended laterally to the thyroid cartilage (figure 2). A workup for systemic amyloidosis was negative. The patient underwent an uneventful left partial supraglottic laser laryngectomy via a transoral route (figure 3). Histologic evaluation confirmed the diagnosis of amyloidosis; both kappa and lambda light chains were identified, although the latter predominated. Five months after treatment, the patient was disease-free (figure 4). Adjuvant radiation and chemotherapy were deferred while the patient underwent close follow-up by laryngoscopy and MRI.

[FIGURES 1-4 OMITTED]

Amyloid is made up of extracellular deposition of fibrillar proteins. It can be found in any organ. Histologically, Congo red stains are used to identify amyloid because of its characteristic apple-green birefringence when viewed under polarized light. On electron microscopy, an interlacing meshwork of nonbranching fibrils is seen.

Amyloidosis can be systemic or localized. The larynx is the most common site of localized disease. Systemic amyloidosis should be ruled out in all patients; the workup includes a complete blood count, a peripheral blood smear, measurement of electrolytes and BUN/creatinine levels, urinalysis, electro- and echocardiography, and a radiographic skeletal survey.

MRI can provide information on the level of involvement. The rapid recurrence in our patient led us to believe that the initial resection most likely had been incomplete--that is, only the polypoid component had been removed. Indeed, on MRI, the extent of disease was completely visualized and the necessity for a larger resection was assessed.

The primary treatment of localized laryngeal amyloidosis is surgical excision. Complete removal affords the greatest opportunity for cure. Other treatments--such as corticosteroids, radiotherapy, and antimetabolites--have yielded only minimal success. When laryngeal amyloidosis is allowed to progress untreated, it may lead to vocal fold fixation, severe dysphonia, and airway obstruction. Endoscopic resection is the treatment of choice. In cases of diffuse laryngeal amyloidosis, surgical resection can result in complete laryngeal stenosis during the healing phase; in such cases, a staged resection should be considered.

Suggested reading

Lebowitz RA, Morris L. Plasma cell dyscrasias and amyloidosis. Otolaryngol Clin North Am 2003;36:747-64.

Sofia Avitia, MD; Jason S. Hamilton, MD; Ryan F. Osborne, MD FACS 

From the Department of Otolaryngology--Head and Neck Surgery, Charles R. Drew University of Medicine and Science (Dr. Avitia); the Osborne Head and Neck Institute (Dr. Hamilton and Dr. Osborne); and the Head and Neck Cancer, Cedars-Sinai Medical Center (Dr. Osborne), Los Angeles.