Primary Localized Laryngeal Amyloidosis: Report of 3 Cases With Long-term Follow-up and Review of the Literature

Archives of Pathology & Laboratory Medicine, Feb 2005 by Ma, Linglei, Bandarchi, Bizhan, Sasaki, Clarence, Levine, Steven, Choi, Young

* Context.-Localized laryngeal amyloidosis is an uncommon condition with limited long-term follow-up studies. The precise etiology and pathogenesis are not entirely clear.

Objective.-To further characterize the histopathologic features and possible pathogenesis of localized laryngeal amyloidosis.

Design.-Three cases of primary localized laryngeal amyloidosis were identified at our institutions from 1980 to 2003. The clinical features and histologie and immunohistochemical patterns were evaluated. Systemic workups were pursued during the long-term follow-up.

Results.-The common presentation of the patients was hoarseness. The lesions involved vocal cords, anterior commissure, and ventricle. Microscopically, the amyloid was present within the submucosa with an adjacent lymphoplasmacytic infiltrate. The plasma cells and amyloid demonstrated monoclonal light chain restriction in all 3 cases (2 λ, 1 κ). No evidence of systemic amyloidosis or an overt B-cell lymphoma was found in these patients. Two patients with long-term follow-up underwent subsequent surgical removals for multiple recurrences, which occurred within 1 year of the initial diagnosis.

Conclusions.-The demonstration of monoclonal light chain expression in the plasmacytic infiltrate and amyloid component in the absence of systemic lymphomas indicates that localized laryngeal amyloidosis may represent a form of benign monoclonal plasma cell dyscrasia. A close follow-up of the patients may be indicated for early detection of recurrences.

(Arch Pathol Lab Med. 2005;129:215-218)

Amyloidosis is a group of disorders in which an extracellular deposition of an abnormal amount of proteins occurs in a variety of organs, including the larynx, von Rokitansky1 first described such deposits in 1842, but it was not until 1851 that Virchow2 applied the term amyloid to describe this deposition. Clinically, amyloidosis is divided into 2 categories: systemic and localized. Approximately 9% to 15% of amyloidosis is of the localized type. Localized amyloidosis in the head and neck is a rare and benign disease. The larynx is the most common site of involvement and accounts for 0.2% to 1.5% of benign Iaryngeal tumors. Within the larynx, the vocal folds and ventricle are more commonly affected. Presenting symptoms include hoarseness, sometimes cough, and sensation of fullness. Stridor and dyspnea may occur in patients with extensive involvement. Laryngeal involvement could be either diffuse subepithelial deposition (diffuse type) or discrete tumor nodules (nodular type).3 Histologically, the presence of amyloid can be confirmed by the characteristic Congo red staining under polarized light microscopy, through immunohistochemical stains, or by electron microscopic findings of laryngeal biopsy specimens. In this report, we examined 3 cases of localized laryngeal amyloidosis in our institutions from 1980 to 2003 and reviewed clinical, pathologic, histochemical, and immunohistochemical aspects of the disease.

REPORT OF caseS

Patient 1 (Table) was a 36-year-old man who presented with several months of hoarseness at the end of 1989. He was neither a smoker nor drinker. His medical history was unremarkable. Laryngoscopic examination at initial presentation revealed small nodular lesions that involved the anterior two thirds of both vocal cords and the anterior commissure. Microdirect laryngoscopy with forceps excision was performed. The lesion was biopsied and diagnosed as laryngeal amyloidosis. All other laboratory findings, including chest x-ray examination, blood workup, and serum and urine protein electrophoresis, were normal. The first recurrence was 1 year later in the beginning of 1991, involving the same location. Later there were multiple recurrences, and the patient underwent subsequent microdirect laryngoscopy and forceps excisions in 1992, 1993, 1997, and 1999. With each additional procedure, the size of the lesion increased from 1.5 to 3.0 cm in greatest dimension. In 2000, the patient care was transferred to one of the authors (S.B.L.), and laryngoscopic examination revealed that the endolarynx was filled with multiple large polypoid lesions that involved the entire supraglottis and anterior commissure, with a compromised endolaryngeal lumen. The lesions were treated with a combination of forceps excision and carbon dioxide laser. Two additional similar procedures were performed in 2000 and again in 2002. During the last procedure, the patient experienced an intraoperative pneumothorax that was treated with a thoracostomy tube and tracheotomy. Six weeks later, the lesion was debulked using a powered laryngeal microdebrider, permitting the surgeon to achieve a more extensive debulking than had previously been performed. The tracheotomy was decannulated, and the site healed without incident. A second debulking with the microdebrider and standard endotracheal intubation was performed 5 months later. No further procedures have been performed in the subsequent 12 months. Despite the repeated recurrences, his disease remains limited to the larynx only (Figure 1).