Abstract
Amyloid is an extracellular proteinaceous low-molecular-weight fibril. In the larynx, amyloid deposits usually represent a benign localized disease. Although laryngeal amyloidosis is an indolent lesion, amyloid deposition in the larynx may actually result from a lymphoproliferative disorder and not isolated amyloidosis. In this case report, we describe a patient referred for laryngeal amyloidosis who was subsequently diagnosed with extramedullary plasmacytoma of the larynx. The report discusses the presentation, diagnosis, and treatment provided. The importance of systemic workup and accurate tissue diagnosis in differentiating primary amyloidosis and secondary amyloid deposition will be highlighted.
1
Introduction
Amyloidosis, the extracellular accumulation of proteinaceous low-molecular-weight fibrils, is generally uncommon in upper aerodigestive structures. Amyloid accounts for 0.1%–1.2% of benign laryngeal lesions . Laryngeal amyloidosis is usually localized and rarely part of systemic disease . However, some cases of laryngeal amyloidosis can be secondary processes resulting from lymphoid neoplastic processes, neuroendocrine carcinomas, or inflammatory conditions, making careful investigation important. In this case report, we describe a patient referred for laryngeal amyloidosis who was subsequently diagnosed with extramedullary plasmacytoma of the larynx. The report discusses the presentation, pathologic diagnosis, and treatment provided. The different prognosis and management for isolated laryngeal amyloidosis and secondary amyloidosis will be highlighted in the discussion. The Johns Hopkins Medical Institution Review Board approved this report.
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Case report
An 80-year-old female was referred for management of laryngeal amyloidosis. She had a 2-year history of progressive hoarseness leading to increased vocal effort and vocal strain. She denied breathing or swallowing difficulties. The referring physician had noted right paraglottic fullness and biopsy revealed laryngeal amyloidosis. The patient’s past medical history includes hypertension, osteoarthritis, obesity, cardiac arrhythmia, and hemithyroidectomy. Work-up for systemic amyloidosis with computed tomography of chest/abdomen/pelvis, serum and urine protein electrophoresis, and immunoelectrophoresis was negative.
Upon presentation to our clinic, laryngeal stroboscopy revealed right paraglottic fullness and convex right vocal fold contour. Ipsilateral mucosal waveforms were greatly reduced, and contact of the convex right vocal fold against the left vocal fold also deformed left mucosal waveforms. Remaining head and neck examination was unremarkable. Initially, she elected conservative follow-up for her isolated voice complaints. As the lesion enlarged on serial exam, and as voice got worse, microlaryngeal surgery for lesion debulking was discussed; unfortunately, the patient was then lost to follow-up for several months secondary to co-morbid conditions. Upon re-presentation, she described increasing exertional dyspnea with rare inspiratory stridor. On stroboscopy, the right-sided fullness had greatly increased in size, with effacement of the right false as well as true vocal cord ( Fig. 1 ).
Microlaryngeal surgery with flexible CO2 laser (OmniGuide, Cambridge, MA) allowed submucosal debulking of disease from the right hemilarynx. A firm mass extended from the right false vocal fold level through the laryngeal ventricle and into the paraglottic space through the true vocal fold’s superior surface. Debulking of the right paraglottic space preserved the true vocal fold medial edge. Pathologic evaluation revealed plasmacytoma with extensive amyloid deposition, CD38-immunoreactivy, and kappa light-chain restriction. Ki-67 immunostaining demonstrated low proliferation index. Congo red staining showed apple-green birefringence under polarized light, characteristic of amyloid deposition ( Fig. 2 ).
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