Abstract
Primary localized laryngeal amyloidosis is a rare presentation of amyloid deposition. A case of 22 years old male presented with progressive hoarseness of voice and supraglottic mass on laryngoscopy reported here. Complete excision of mass was done using Coblator wand. Histopathological examination and Congo red stain demonstrated AL amyloidosis of Larynx.
Keywords: Amyloidosis, Larynx, Hoarseness of voice, Recurrence
Introduction
Amyloidosis is a group of disorders caused by extracellular deposition of insoluble fibrillar protein (amyloid) with characteristic features on microscopy, histochemistry and ultrastructural examination. This deposition of amyloid can be localized or systemic and interferes with the function of target organ. Localized amyloidosis although rare in incidence has better outcome as compared to systemic amyloidosis.
Burrow and Neumann in 1875 reported first case of Laryngeal amyloidosis [1]. Localized Laryngeal amyloidosis is extremely rare, accounts for 0.2–1.2% of all benign tumours of larynx although it is the most commonly affected organ by amyloidosis in Head Neck region. In the larynx, various sites involved in decreasing order of frequency are the false cords, aryepiglottic fold and subglottic region [2]. It has higher male predilection with 3:1 male -female ratio and its peak incidence in fifth to sixth decade [3]. Dysphonia is a commonest presenting symptom. We report a case of isolated laryngeal amyloidosis with hoarseness of voice with a aim to highlight the need of high index of suspicion from ENT Surgeon.
Case Report
A 22 years old male patient reported with gradually progressive hoarseness of voice for 6 months without dysphagia, dyspnoea or stridor. He was a chronic Ghutka chewer but no alcohol intoxication and without any significant family history. The patient had no known autoimmune disease, chronic inflammatory condition or any known malignancy. On clinical examination, patient’s voice was severely hoarse and strained. Laryngoscopic examination revealed submucosal firm bosselated mass involving left false cord, ipsilateral arytenoid and ventricle (Fig. 1). Vocal cords and subglottis were normal. Biopsy was taken from the growth with a suspicion of malignancy. Histopathological examination revealed deposition of acellular, homogenous, amorphous and eosinophilic deposits within the stroma. Congo Red staining showed apple green birefringence characteristic of amyloidosis (Fig. 2). No dysplasia or malignant cells were detected.
Fig. 1.
Endoscopic view of lesion involving left supraglottis
Fig. 2.
Apple green birefringence with Congo red staining
Patient was further evaluated with a concern for systemic amyloidosis. His comprehensive medical work up including Complete Blood counts, Erythrocyte Sedimentation Rate, Serum Calcium, Sodium and Potassium, Renal and Liver Function Tests, Serum Immunoglobulin Level were normal. Serum and Urine Protein Electrophoresis did not show any monoclonal protein. ECG, 2D Echocardiogram, Ultrasonography of abdomen, X-ray Chest were normal. Investigations to rule out chronic inflammatory conditions including rheumatological condition were normal indicating no systemic involvement.
A complete resection of laryngeal lesion was done by Micro-laryngeal surgery using Coblator laryngeal wand. Postoperatively there was subsequent improvement in the quality of voice through speech therapy. Patient is under regular follow-up since last 6 months with no evidence of systemic amyloidosis or local recurrence of the disease.
Discussion
Amyloidosis is a rare benign disease characterized by extracellular deposition of proteinaceous material in the variety of tissues, which has typical staining properties and electron microscopic appearance. Its exact etiology is still unclear. Theory proposed is that amyloid light chains are produced by plasma cell clones. Another theory is its occurrence secondary to body’s inability to remove light chain proteins leading to localized deposits [3].AL amyloidosis, the most common form amongst 36 types can be systemic or localized and preferentially impacts the cardiovascular, renal, and hepatic systems [4].
Amyloidosis of head and neck can be of two types, a primary isolated disease or secondary to systemic involvement. Isolated laryngeal amyloidosis is very rare. Other sites that can get involved are orbits, the salivary glands and upper aerodigestive tract. Hoarseness of voice is the most common presentation of Laryngeal amyloidosis but sometimes patients present with dyspnea, stridor, persistent dry cough, sleeping apnea syndrome or dysphagia. On laryngoscopy, lesion may vary from nodular form, polypoidal to diffuse infiltration. This may falsely suggest a neoplastic disease, fibroma, papilloma or tuberculosis. Systemic involvement should also be excluded in each patient as localized and systemic amyloidosis have completely different prognosis and management. The goal of the treatment for localized Laryngeal Amyloidosis is to provide disease free interval to the patient as long as possible with voice preservation and normal breathing. Treatment may vary from simple observation, more conservative endoscopic excision to radical excision by partial laryngectomy depending upon the extent of involvement of larynx. Endoscopic excision can be done either with cold knife, CO2 LASER, Microdebrider or with Coblation wands [5]. In our case, we excised the lesion using Coblator with Laryngeal wand. Medical treatment with colchicine found to be complementary to surgery [6]. Wadhawa A et al. found effective result with oral Bortezomib and corticosteroid after debulking diffuse laryngeal amyloidosis [7].
The overall prognosis of Laryngeal Amyloidosis is generally good, but the probability of recurrent or residual disease is significant and death due to respiratory failure has been reported [8]. It is more common in first 5 years after treatment [9]. Mesollela M et al. in their case series performed monthly check-ups for the first 6 months, bimonthly until the end of the second year, then six-monthly for two more years and yearly from the fifth year onwards and recommended at least 10 years laryngological follow-up [10].
The proposed theory is that recurrence of the disease slows over time due to exhaustion of underlying clonal plasma cells [9].
Conclusion
Localized Laryngeal amyloidosis is a rare cause for hoarseness of voice. Confirmation of diagnosis by tissue biopsy with Congo Red staining and evaluation for systemic involvement is essential. Surgical excision is the treatment of choice and CO2 LASER has been the preferred modality. But according to recent literature and in our experience Coblation wand is an effective option in its management. Long term follow-up is essential to in this condition because of high local recurrence rate reported in the literature.
Authors’ Contributions
Authors analyzed the patient’s medical data regarding the disease and performed the treatment. They were also involved in conception and design of the manuscript and drafting it.
Funding
None.
Declarations
Conflict of interest
The authors declare that they have no conflict of interests.
Consent to Publication
This case report has never been published and is not currently under evaluation in any other peer reviewed publication.
Informed Consent
Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review.
Footnotes
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