Laryngeal Amyloidosis

Abstract

Objectives: Amyloidosis constitutes less than 1% of benign laryngeal tumors and may occasionally be associated with systemic disease. It commonly presents with long-standing hoarseness or dyspnea. Diagnosis is established with histological features of acellular, amorphous, eosinophilic deposits which react positively with Congo red. We present 2 such cases of laryngeal amyloidosis, one localized and one with systemic involvement. Literature review, diagnostic work-up, and treatment options are discussed. Methods: Case 1: A 63 year old non-smoking male presented with hoarse voice for 2 months. Flexible laryngoscopy showed diffuse fullness on left hemilarynx. Microlaryngoscopy revealed submucosal lesion in anterior left vocal cord, which on deep biopsy confirmed AL type amyloid. Detailed work-up by a hematologist ruled out systemic disease. Case 2: A 74 year old moderate smoker presented with 6 months of hoarse voice and burning sensation in the tongue. He had a mucosa-covered growth in the right false vocal cord, which on biopsy confirmed amyloidosis (AL type). Further work-up revealed systemic disease with elevated serum free light chains, IgG Kappa paraprotein, and positive abdominal fat biopsy. Results: Patient 1 is under follow-up with no clinical recurrence. Patient two had treatment with CDT chemotherapy and is under hematologist surveillance. Past history includes carpal-tunnel decompression. Localized laryngeal amyloidosis is best treated with cold steel resection or CO2 laser excision and may require repeated removal in some patients. Systemic amyloidosis is caused by plasma-cell dyscrasia and treated with chemotherapy and or radiotherapy. Conclusions: Endolaryngeal treatment is sufficient with localized disease. Systemic disease needs more extensive treatment, and those with associated myeloma have poor prognosis.