Amyloidosis of the larynx: A clinicopathologic study of 11 cases

Laryngeal amyloidosis (LA) is uncommon and poorly understood, with limited long-term clinicopathologic and immunophenotypic studies in the Literature. Eleven cases of LA mere retrieved from the files of the Otorhinolaryngic-H ead & Neck Tumor Registry from 1953 to 1990. The histology, histochemistry, immunohistochemistry, and follow-up were reviewed. All patients (three wom en and eight men) presented with hoarseness at an average age of 37.8 years . The lesions, polypoid or granular, measured an average of 1.6 cm and invo lved the true vocal cords only (n = 4), false vocal cord only (n = 1), or w ere transglottic (n = 6), An acellular, amorphous, eosinophilic material wa s present in the stroma, often accentuated around vessels and seromucous gl ands, which reacted positively with Congo red, A sparse lymphoplasmacytic i nfiltrate was present in all cases that demonstrated light chain restrictio n by immunohistochemistry in three cases (kappa = 21 lambda = 1). Serum and urine electrophoreses were negative in all patients. Treatment was Limited to surgical excision, including a single laryngectomy, Six patients manife sted either recurrent and/or multifocal/systemic disease: two patients with light chain restriction were dead with recurrent disease (mean, 11.1 years ); two patients were dead with no evidence of disease (mean, 31.7 years); a nd two patients were alive, one with light chain restriction and recurrent and multifocal disease (41.6 years) and one with no evidence of disease aft er a single recurrence (43.4 years). The remaining five patients were eithe r alive or had died with no evidence of disease an average of 32.4 years af ter diagnosis. No patient developed multiple myeloma or an overt B-cell lym phoma. LA is an uncommon indolent lesion that may be associated with multifocal di sease (local or systemic). The presence of an associated monoclonal lymphop lasmacytic infiltrate and recurrent/multifocal disease in the respiratory o r gastrointestinal tract of a few cases and the lack of development of a sy stemic plasma cell dyscrasia or overt systemic B-cell malignancy suggest th at some LA may be the result of an immunocyte dyscrasia or tumor of mucosa- associated lymphoid tissue.