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CC BY-NC-ND 4.0 · Int Arch Otorhinolaryngol 2020; 24(04): e450-e456
DOI: 10.1055/s-0039-3402494
Original Research

Clinical Profile of Patients with Head and Neck Amyloidosis: A Single-Institution Retrospective Chart Review

Anup Singh 
1   Department of Otorhinolaryngology and Head and Neck Surgery, Medanta - The Medicity, Gurugram, Haryana, India
,
Mubashshirul Haq
1   Department of Otorhinolaryngology and Head and Neck Surgery, Medanta - The Medicity, Gurugram, Haryana, India
,
Poonam Gautam
1   Department of Otorhinolaryngology and Head and Neck Surgery, Medanta - The Medicity, Gurugram, Haryana, India
,
Dheeraj Gautam
2   Department of Pathology, Medanta - The Medicity, Gurugram, Haryana, India
,
Aru C. Handa
1   Department of Otorhinolaryngology and Head and Neck Surgery, Medanta - The Medicity, Gurugram, Haryana, India
,
Kumud K. Handa
1   Department of Otorhinolaryngology and Head and Neck Surgery, Medanta - The Medicity, Gurugram, Haryana, India
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Abstract

Introduction Isolated amyloidosis involving the head and neck is a rare entity. The pathophysiology of the localized disease appears to be distinct from that of the systemic counterpart. Systemic progression of the localized disease is unusual, and the prognosis of the localized form is excellent.

Objective To describe the demographic and clinicopathological characteristics of patients presenting with localized head and neck subsite amyloidosis.

Methods A retrospective chart review of the patients with head and neck amyloidosis identified by the electronic search of the electronic database of the Departments of Pathology and Otorhinolaryngology was performed. The various demographic and clinical data were tabulated.

Results In total, seven patients (four females, three males) with localized head and neck amyloidosis (three supraglottic, three lingual and one sinonasal) were identified. Six patients had AL-amyloid deposits, and one patient had AA-amyloid deposits. Supraglottic involvement and that of the base of the tongue were treated surgically using CO2 laser, and these patients were disease-free at the last follow-up. The patient with sinonasal amyloidosis experienced symptom recurrence after six months of the functional endoscopic sinus surgery. All of the patients were screened for systemic amyloidosis with abdominal fat pad biopsy, and were found to be free of systemic spread.

Conclusion Isolated head and neck amyloidosis, as opposed to systemic amyloidosis, has an excellent prognosis in terms of survival. Therefore, systemic amyloidosis should be excluded in all cases. The treatment of choice remains surgical excision; however, watchful waiting may be a suitable strategy for mild symptoms or for cases in which the disease was discovered incidentally.

Keywords

amyloidosis - plasma cells - laryngeal diseases - macroglossia - prognosis

Declarations

• The present work was performed at Medanta - The Medicity, Gurugram, Haryana, India.


• Informed/Written consent was waived by the Institutional Review Board.




Publication History

Received: 04 September 2019

Accepted: 11 November 2019

Article published online:
07 February 2020

© .

Thieme Revinter Publicações Ltda
Rio de Janeiro, Brazil

 

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