Amyloidosis is the deposition of an extracellular fibrillar protein in the tissues leading to organ dysfunction. Laryngeal amyloidosis is a rare phenomenon. We report a case of isolated laryngeal amyloidosis which was initially suspicious for laryngeal cancer on magnetic resonance imaging (MRI) but histopathology showed the presence of amyloid. Systemic workup was negative. The patient is being managed conservatively

Gupta,, Supriya

Muneeb, Aeman

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eCommons@AKUDepartment of Radiology Medical College, PakistanAugust 2018Isolated laryngeal amyloidosis mimicking laryngealcancerAeman MuneebAga Khan UniversitySupriya Gupta,University Medical Center, Chicago , USA.Follow this and additional works at: https://ecommons.aku.edu/pakistan_fhs_mc_radiolRecommended CitationMuneeb, A., Gupta,, S. (2018). Isolated laryngeal amyloidosis mimicking laryngeal cancer. Cureus, 10(8), 1-4.Available at: https://ecommons.aku.edu/pakistan_fhs_mc_radiol/320Received 06/22/2018 Review began 06/25/2018 Review ended 08/03/2018 Published 08/06/2018© Copyright 2018Muneeb et al. This is an open accessarticle distributed under the terms ofthe Creative Commons AttributionLicense CC-BY 3.0., which permitsunrestricted use, distribution, andreproduction in any medium, providedthe original author and source arecredited.Isolated Laryngeal Amyloidosis MimickingLaryngeal CancerAeman Muneeb  , Supriya Gupta 1. Medical College, The Aga Khan University, Karachi, PAK 2. Radiology, Rush University Medical Center,Chicago , USA Corresponding author: Aeman Muneeb, aemanmuneeb@live.com Disclosures can be found in Additional Information at the end of the articleAbstractAmyloidosis is the deposition of an extracellular fibrillar protein in the tissues leading to organdysfunction. Laryngeal amyloidosis is a rare phenomenon. We report a case of isolatedlaryngeal amyloidosis which was initially suspicious for laryngeal cancer on magneticresonance imaging (MRI) but histopathology showed the presence of amyloid. Systemic workupwas negative. The patient is being managed conservatively.Categories: Otolaryngology, RadiologyKeywords: laryngeal amyloidosisIntroductionExtracellular deposition of an abnormal fibrillar protein in the tissues is termed amyloidosis [1].The incidence of amyloidosis ranges from 5 to 13 per million per year individuals worldwide [2].Diagnosis requires histological examination of the tissue with Congo red stain [1].Amyloidosis most commonly affects men around the age of 50 to 70 years [3]. Treatment isbased on timely identification and therapies are aimed at reducing the production of newamyloid protein by limiting the supply of amyloid precursor protein [1].In the head and neck, the larynx is one of the frequent sites of amyloid deposition [3]. It mayinvolve true vocal cords, false vocal cords or may be transglottic [4]. Isolated laryngealamyloidosis is a rare phenomenon and comprises about 0.2% to 1.2% of all benign laryngealtumors [3] Even though laryngeal amyloidosis is a localized process, it may be associated withsystemic disease, and the recurrent/persistent disease appears to be the norm rather than theexception [4]. The laryngoscopy findings usually demonstrate a yellowish-white mass arisingfrom the larynx [3,5]. Our case report emphasizes the importance of keeping a high suspicionfor laryngeal amyloidosis in a patient with atypical presentation of hoarseness especially slow-growing masses, with characteristic findings on laryngoscopy.Case PresentationA 31-year-old male presented with stable dysphonia for four years. There were no sinonasalcomplaints, dyspnea, dysphagia, sore throat or a cough. Direct laryngoscopy exam showed leftsupraglottic/false vocal fold submucosal fullness extending to the aryepiglottic fold withpapillomatous changes to the mucosa of the false vocal fold (Figure 1). Magnetic resonance imaging (MRI) of the neck revealed an ill-defined enhancing soft tissue1 2 Open Access CaseReport  DOI: 10.7759/cureus.3106How to cite this articleMuneeb A, Gupta S (August 06, 2018) Isolated Laryngeal Amyloidosis Mimicking Laryngeal Cancer.Cureus 10(8): e3106. DOI 10.7759/cureus.3106mass centered in the left false vocal cord and paraglottic fat, extending to the left true vocalcord, anterior commissure, and left aryepiglottic fold (Figure 2). Imaging findings weresuspicious for laryngeal cancer. No pathologically enlarged lymph nodes were identified by theimaging size criteria. The pathology report showed congophilic amyloid deposits within thestroma and around the sub-mucosal glands containing a mixed population of kappa andlambda staining plasma cells. The findings were consistent with laryngeal amyloidosis.Systemic workup including serum and urine electrophoresis, cardiac exam, and bone marrowbiopsy was negative. Local debridement was considered; however, due to the disease size andconcern for residual amyloidosis, the patient was managed conservatively. Follow-up MRIperformed three months later showed no interval change.FIGURE 1: Laryngoscopy findingsLeft supraglottic/false vocal fold submucosal fullness (arrow) extending to the aryepiglottic fold withpapillomatous changes to the mucosa of the false vocal fold.2018 Muneeb et al. Cureus 10(8): e3106. DOI 10.7759/cureus.3106 2 of 4FIGURE 2: Axial T1 post-contrast magnetic resonance imaging(MRI)Ill-defined enhancing soft tissue mass (arrow) centered in the left false vocal cord and paraglotticfat, extending to the left true vocal cord, anterior commissure, and left aryepiglottic fold.DiscussionTwo possible mechanisms have been hypothesized leading to the development of isolatedlocalized amyloidosis [3]. The first mechanism hypothesizes abnormal proliferation anddeposition of plasma cell clones that produce amyloid light chain protein. The secondmechanism focuses on the body’s inability to clear light chain proteins resulting in localizeddeposition.The most common presentation of laryngeal amyloidosis is hoarseness, even though a cough,hemoptysis, dysphagia or dyspnea may be present [3-4]. A review of 11 cases [4] found that allcases presented with the chief complaint of hoarseness similar to our case. MRI findings weresuspicious for laryngeal cancer but pathology report showed amyloidosis. It has been reportedthat MRI is a generally reliable tool to diagnose amyloidosis since amyloid gives anintermediate T1 signal and low T2 signal [3]. However, since amyloidosis may mimic cancer,the clinician must have a reasonable degree of suspicion and confirm the diagnosis with abiopsy [6].Laryngeal amyloidosis is most often treated surgically [4]; however, in our case, surgery wasavoided due to concerns for incomplete excision and residual disease. Conservativemanagement was undertaken. Recurrent disease is common with a good 10-year overallsurvival after initial presentation [4]. Some patients may spontaneously go into remission withno evidence of disease years after diagnosis [4].New therapies such as anti-amyloid antibodies are under trial for systemic disease. These have2018 Muneeb et al. Cureus 10(8): e3106. DOI 10.7759/cureus.3106 3 of 4the ability to dissolve amyloid deposits [7] thus reducing the population of monoclonal plasmacells responsible for amyloid production.ConclusionsAmyloidosis of the larynx is a rare disease, often presenting with hoarseness. Clinical suspicionfor this entity should be high in young patients with slow-growing laryngeal mass. Treatmentcurrently consists of surgical resection though newer therapies for amyloidosis may lead tobetter outcomes.Additional InformationDisclosuresHuman subjects: Consent was obtained by all participants in this study. Conflicts of interest:In compliance with the ICMJE uniform disclosure form, all authors declare the following:Payment/services info: All authors have declared that no financial support was received fromany organization for the submitted work. Financial relationships: All authors have declaredthat they have no financial relationships at present or within the previous three years with anyorganizations that might have an interest in the submitted work. Other relationships: Allauthors have declared that there are no other relationships or activities that could appear tohave influenced the submitted work.References1. Gillmore JD, Hawkins PN: Pathophysiology and treatment of systemic amyloidosis . Nat RevNephrol. 2013, 9:574-586. 10.1038/nrneph.2013.1712. Nienhuis HL, Bijzet J, Hazenberg BP: The prevalence and management of systemicamyloidosis in western countries. Kidney Dis. 2016, 2:10-19. 10.1159/0004442063. Behnoud F, Baghbanian N: Isolated laryngeal amyloidosis. Iran J Otorhinolaryngol. 2013,25:49-52.4. Thompson LDR, Derringer GA, Wenig BM: Amyloidosis of the larynx: a clinicopathologicstudy of 11 cases. Mod Pathol. 2000, 13:528. 10.1038/modpathol.38800925. Wang Q, Chen H, Wang S: Laryngo-tracheobronchial amyloidosis: a case report and review ofliterature. Int J Clin Exp Pathol. 2014, 7:7088-7093.6. Siddachari RC, Chaukar DA, Pramesh CS, Naresh KN, de Souza CE, Dcruz AK: Laryngealamyloidosis. J Otolaryngol. 2005, 34:60-63.7. Zumbo G, Sadeghi-Alavijeh O, Hawkins PN, Fontana M: New and developing therapies for ALamyloidosis. Expert Opin Pharmacother. 2017, 18:139-149. 10.1080/14656566.2016.12749712018 Muneeb et al. Cureus 10(8): e3106. DOI 10.7759/cureus.3106 4 of 4

Isolated laryngeal amyloidosis mimicking laryngeal cancer

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Isolated laryngeal amyloidosis mimicking laryngeal cancer

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