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
