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

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; laryngeal diseases; macroglossia; plasma cells; prognosis.

Fig. 1

(A) Microlaryngoscopic view showing the mucosa-covered bulge involving bilateral true vocal cords (*). (B) Excision of the lesion using CO ₂ laser in the process. (Patient-1)

Fig. 2

Non-contrast computed tomography (CT) scan of the paranasal sinuses: (A) coronal and (B) axial views showing bulky soft-tissue density involving the bilateral nasal cavity, the middle meatus and the anterior ethmoid region, causing expansion of the nasal cavity with lateral displacement of the lateral nasal wall. The ‘fluffy’ bony changes, most pronounced in the inferior turbinate bone, can be appreciated (arrows) . (Patient-7)

Fig. 3

(A; B) Light microscopic view showing homogenous extracellular eosinophilic amorphous subepithelial and perivascular deposition of amyloid ([A] hematoxylin and eosin stain; [B] Congo red stain, non-polarized; original magnification: 100x). (C) Polarized microscopy showing apple-green birefringence with Congo red corresponding to areas of amyloid deposition. (Original magnification: 100x.)