International Journal of Head and Neck Surgery
Volume 14 | Issue 4 | Year 2023

Isolated Laryngotracheal Amyloidosis with Rare Clinical Presentation and Latest Surgical Management: Case Report and Review of Literature

Anupama Satpathy1, Arjun Dasgupta2, Chirajit Dutta3, Nittala Venkata K Mohan4

1–4Department of ENT and Head Neck Surgery, CK Birla, CMRI Hospital, Kolkata, West Bengal, India

Corresponding Author: Anupama Satpathy, Department of ENT and Head Neck Surgery, CK Birla, CMRI Hospital, Kolkata, West Bengal, India, Phone: +91 9051732751, e-mail:

Received: 21 January 2022; Accepted: 27 September 2023; Published on: 16 January 2024


Isolated laryngotracheal amyloidosis is a rare disorder, accounting for only 0.2–1.2% of benign masses of the larynx. In the larynx, the rarest site mentioned in literature for amyloidosis is the subglottis and tracheal wall. Here, we present a case of a 69-year-old male who complained of progressive hoarseness, was diagnosed with subglottis and tracheal wall amyloidosis, and was treated with endoscopic microlaryngeal surgery and excision with coblation. As it is a slowly progressive disease, long-term follow-up is always recommended.

How to cite this article: Satpathy A, Dasgupta A, Dutta C, et al. Isolated Laryngotracheal Amyloidosis with Rare Clinical Presentation and Latest Surgical Management: Case Report and Review of Literature. Int J Head Neck Surg 2023;14(4):68–70.

Source of support: Nil

Conflict of interest: None

Keywords: Amyloidosis, Case report, Coblation, Hoarseness, Larynx, Tracheal


Amyloidosis is a Greek word meaning “resemblance to starch.” Virchow coined the term amyloidosis.1 It is a rare disorder of extracellular hyaline fibrillar material deposition in a few target organs such as the heart, liver, kidneys, nervous system, etc. It is very slowly progressing but leads to organ dysfunction and then failure.2 Localized amyloidosis in the head and neck is even rarer than systemic amyloidosis. Localized laryngeal amyloidosis accounts for only 0.2–1.2% of benign masses of the larynx. First case of laryngeal amyloidosis was detected in 1842 in a postmortem examination. Most common age group affected is 50–70 years, and male:female predisposition is 3:1. In the literature, localization of lesion in larynx is to the ventricle, false vocal cords, true vocal cords, aryepiglottic folds, and subglottis. Most common presenting symptom in laryngeal amyloidosis is hoarseness, followed by dysphagia, exertional dyspnea, rarely hemoptysis, or airway obstruction.3 Histochemical analysis is the mainstay in diagnosis, and endoscopic or microlaryngeal surgery and carbon dioxide (CO2) laser or coblation excision are the mainstay of treatment.4 Here, we are presenting a case of laryngotracheal amyloidosis involving subglottis and tracheal wall with presenting symptoms, diagnosis, and advanced surgical management.


A male patient of 69 of years age presented at the outpatient department with progressive hoarseness for 1–1.5 years. There was no history of dyspnea, stridor, postnasal drip, dysphagia, or hemoptysis. He gave negative history of trauma, weight loss, anorexia, night sweats, joint pains, fatigability, recurrent fever, abdominal pains, or chest pains. He has been known to be hypertensive for around 1.5 years and has had diabetes mellitus (type II) for the last 6–7 months [after he was treated for coronavirus disease 2019 (COVID-19) pneumonia in May 2021]. Both comorbidities are under control with regular oral hypoglycemics and antihypertensive medications. He was a heavy smoker for the last 30–40 years but quit for the last 1 year. There was only one episode of hospitalization in all these years for COVID-19 in May 2021, and that was only for observation and medical management for around 20 days without any need for endotracheal intubation and ventilatory support.

On examination of the oral cavity, he had average orodental hygiene and stained teeth. No growth or ulcer was detected in the oral cavity and oropharynx. Neck examination revealed normal laryngeal crepitus and no significant cervical lymphadenopathy. Then, after explaining the patient, he was taken for fiberoptic laryngoscopy, which showed a smooth, pale pinkish granulomatous lesion in the subglottis (Fig. 1). Suspecting papillomatous growth, various granulomatous diseases, or malignancy, he was advised to undergo a computed tomography (CT) scan of the neck. The scan revealed irregular mural thickenings in the subglottic region of the larynx and, caudally, in parts of the trachea (Figs 2 and 3).

Fig. 1: Fiberoptic laryngoscopy showing smooth, pale pinkish granulomatous lesion in subglottis

Fig. 2: Axial section CT scan neck showing mass lesion at the level of subglottic larynx

Fig. 3: Axial section CT scan showing thickening of tracheal wall

His systemic examination, including routine blood tests like complete blood counts, coagulation profile, liver and renal function test, serum immunoglobulin E, urine examination, ultrasonography of the whole abdomen, electrocardiogram, echocardiography, and chest X-ray, were within normal range, except for slightly raised blood sugar and glycated hemoglobin levels reflecting deranged glycemic control.

After reviewing the CT scan of the neck, all reports, and anesthetic clearance, he was taken for microlaryngoscopy and excision of the lesion using coblation. Rigid bronchoscopy and excision of tracheal lesions in the posterior wall, around 4–5 cm above the carina, were also done in the same sitting. Tissue bits from lesion were sent for histopathologic examination. There was not much bleeding peroperatively with coblation, and the patient was extubated and shifted to ward, being stable, and was discharged the next day. After 10 days, the patient came for follow-up, and his voice was improved. Repeat fiberoptic laryngoscopic examination showed evidence of healing with healthy granulation tissue. Histopathological examination diagnosed the case as laryngeal amyloidosis.


Amyloidosis is a very slowly progressing clinical metabolic disorder characterized by extracellular accumulation of abnormal fibrillar protein (amyloid), usually produced in the bone marrow and deposited in various organs, leading to alteration of the normal functions of affected organs. It is a rare disease process, mostly systemic, involving organs such as heart, kidneys, liver, nervous system, and spleen but rarely can be isolated in head and neck. Incidence of amyloidosis is around 5–10 cases per million per year, in which nearly 20% involves the head and neck. Localized laryngeal amyloidosis is a very rare process and comprises only 0.2–1.2% of all benign laryngeal growths. Amyloidosis is a life-threatening condition as it leads to progressive organ dysfunction.2,5 In our case, it was slowly progressive, but as the airway was patent, it did not lead to an emergency.

Laryngeal amyloidosis is a benign, slowly progressive disease. Male:female predisposition is 3:1. It usually affects individuals in the age group of 50–70 years. Clinical presentation of the patient includes hoarseness usually, but it can also present with dyspnea, stridor, hemoptysis, or globus depending on the part and extent of larynx involved. In order of frequency, the most common sites involved in the larynx as per order of frequency are supraglottis (54.05%) involving the ventricle and false vocal cords, glottis (18.91%), tranglottic (16.21%), lastly followed by subglottis and tracheal wall (10.8%). In our case, the lesion was in subglottis and posterior tracheal wall with complaints of hoarseness only.1,6-8

In the literature, two theories for the explanation of localized amyloidosis in the larynx have been proposed. First is secondary amyloid or amyloid of chronic inflammation theory, where AA amyloid deposits with plasma cells generate inflammatory antigens. Second, which is a more acceptable theory, is due to the inability of the body to clear light chains amyloid leading to its deposition with plasma cells located in mucosal-associated lymphoid tissue (AL amyloid).9 Histochemically, amyloidosis can be broadly divided into two types—(1) primary or myeloma-associated amyloidosis, where fibril protein is a light chain immunoglobulin designated as AL, and (2) secondary amyloidosis, which is caused by various inflammatory diseases or rheumatologic conditions and designated as AA amyloidosis.9,10

Diagnosis of laryngotracheal amyloidosis starts with clinical suspicion followed by laryngoscopic examination, then radiological confirmation by CT scan or magnetic resonance imaging. On laryngoscopy, the laryngeal amyloidosis lesion appears as a firm, nonulcerated, smooth, waxy, submucosal nodule, or polypoid lesion that may be yellowish-gray or orange, typically on epiglottis (near ventricle) but sometimes can involve glottis or subglottis.11 In our case, the presentation of the lesion was a bit different. It was yellowish smooth with a pinkish granular surface. There were no ulcerations or bleeding from the lesion. Systemic examinations are done to rule out systemic involvement.

Histological examination of the tissue confirms the diagnosis. Special stains like Congo red are used to confirm amyloidosis, which turns amyloid tissue to reddish-orange on microscopy, then examination under a polarized microscope shows apple green birefringence.12 In our case, histopathology of tissues from subglottis to tracheal wall showed nodular masses of extracellular, homogenous, structureless, and eosinophilic hyaline material on hematoxylin and eosin (H&E) stain. The deposition is noted among degenerated bluish cartilaginous tissue; no granuloma was seen. A special stain with Congo red showed brick-red to orange discoloration of eosinophilic deposits—consistent with subglottic and tracheal amyloidosis (Fig. 4).

Fig. 4: Histological examination showing Congo red deposits of amyloid among degenerated bluish cartilaginous tissue

Treatment of choice for localized laryngotracheal amyloidosis is endoscopic surgical excision either with cold knife or with CO2 laser or both combined.13,14 As the goal for treatment of localized laryngotracheal amyloidosis is to provide better voice, avoid inevitable progression, and patent airway, and we preferred coblation over CO2 laser. Peroperatively, coblation acts as electrosurgical unit, causing coagulation of small vessels, resulting in better hemostasis and providing a bloodless field. Lesser rise in temperature in coblation leads to minimal charring and the least damage to surrounding tissues;14,15 there is lesser postoperative edema, early decannulation of patient, and less chances of reintubation due to airway edema in the postoperative period. In our case, patient was extubated after the completion of the procedure, was stable in the ward overnight, and was discharged the next day. Nonsurgical but not very effective alternative treatment options for laryngotracheal amyloidosis include local steroid injections, systemic steroids, local radiotherapy, and chemotherapy.

Patients should be followed up every 3–6 months to monitor recurrence for the first 5 years and yearly thereafter. Systemic recurrence is very uncommon in this.16


Localized laryngotracheal amyloidosis, especially involving subglottis and tracheal wall, is a rare etiology of hoarseness of voice. Considering its progressive nature and potential to cause severe airway obstruction, laryngeal amyloidosis should be kept as a differential diagnosis in patients presenting with progressive hoarseness of voice. After a thorough evaluation of local sites and confirmation with histopathological and histochemical studies, surgical removal remains the mainstay of management. Ruling out systemic involvement of the disease is also essential before taking decision of final treatment. Though historically, surgical excision with CO2 laser has been preferred, recent case reports and our experience showed removal and ablation of lesion with coblation is definitely superior in terms of less operative time, minimal peroperative bleeding, less postoperative airway complications, speedy recovery, and likely less chances of recurrence. Patients should be on follow-up regularly for at least 5 years, considering the high rate of locoregional recurrence.


1. Walker PA, Courey MS, Ossoff RH. Staged endoscopic treatment of laryngeal amyloidosis. Otolaryngol Head Neck Surg 1996;114(6);801–805. DOI: 10.1016/S0194-59989670105-5

2. Sipe JD, Benson MD, Buxbaum JN, et al. Amyloid fibril proteins and amyloidosis: chemical identification and clinical classification international society of amyloidosis 2016 nomenclature guidelines. Amyloid 2016;23(4):209–213. DOI: 10.1080/13506129.2016.1257986

3. Chow V, Gardner K, Howlett D. Primary localized laryngeal amyloidosis presenting with dysphonia: a case report. J Surg Case Rep 2012;2012(11):rjs005. DOI: 10.1093/jscr/rjs005

4. Alaani A, Warfield AT, Pracy JP. Management of laryngeal amyloidosis. J Laryngol Otol 2004;118(4):279–283. DOI: 10.1258/002221504323012021

5. Hazenberg BP, Van Gameren II, Bijzet J, et al. Diagnostic and therapeutic approach of systemic amyloidosis. Neth J Med 2004;62(4):121–128. PMID: 15255082.

6. Yiotakis I, Georgolios A, Charalabopoulos A, et al. Primary localized laryngeal amyloidosis presenting with hoarseness and dysphagia: a case report. J Med Case Rep 2009;3:9049. DOI: 10.4076/1752-1947-3-9049

7. Behnoud F, Baghbanian N. Isolated laryngeal amyloidosis. Iran J Otorhinolaryngol 2013;25(70):49–52. PMID: 24303419.

8. Fernandes CM, Pirie D, Pudifin DJ. Laryngeal amyloidosis. J Laryngol Otol 1982;96(12):1165–1175. DOI: 10.1017/s0022215100093658

9. Kourelis TV, Kyle RA, Dingli D, et al. Presentation and outcomes of localized immunoglobulin light chain amyloidosis: the mayo clinic experience. Mayo Clin Proc 2017;92(6):908–917. DOI: 10.1016/j.mayocp.2017.02.016

10. Lewis JE, Olsen KD, Kurtin PJ, et al. Laryngeal amyloidosis: a clinicopathologic and immunohistochemical review. Otolaryngol Head Neck Surg 1992;106(4):372–377. DOI: 10.1177/019459989210600410

11. Phillips NM, Matthews E, Altmann C, et al. Laryngeal amyloidosis: diagnosis, pathophysiology and management. J Laryngol Otol 2017:131(S2):S41–S47. DOI: 10.1017/S0022215117000780

12. Biewend ML, Menke DM, Calamia KT. The spectrum of localized amyloidosis: a case series of 20 patients and review of the literature. Amyloid 2006;13(13):135–142. DOI: 10.1080/13506120600876773

13. Behranwala KA, Ali Asgar B, Borges A, et al. Laser in treatment of laryngeal amyloidosis. Indian J Otolaryngol Head Neck Surg 2004;56(1):46–48. DOI:10.1007/BF02968774

14. Deviprasad D, Pujary K, Balakrishnan R, et al. KTP laser in laryngeal amyloidosis: five cases with review of literature. Indian J Otolaryngol Head Neck Surg 2013;65(Suppl 1):36–41. DOI: 10.1007/s12070-011-0435-z

15. Zhang QF, Zhang JJ, Zhang Y, et al. Endoscopic coblation assisted arytenoidectomy in the treatment of bilateral vocal cord paralysis. Zhonghua Er Bi Yan Hou Tou Jing Wai Ke Za Zhi 2013;48(7):589–591. PMID: 24313210.

16. Hazenberg AJ, Hazenberg BP, Dikkers FG. Long-term follow-up after surgery in localized laryngeal amyloidosis. Eur Arch Otorhinolaryngol 2016;273(9):2613–2620. DOI: 10.1007/s00405-016-4061-y

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