Background: Primary laryngeal amyloidosis is extremely rare and represents less than 1% of all benign laryngeal tumours. This is the third reported case of isolated primary epiglottic amyloidosis in the literature.
Case Report: A 54-year-old female presented to the head and neck surgical oncology clinic with an incidental epiglottic lesion found during intubation for an unrelated surgical procedure. She is an ex-smoker and was otherwise well. She was asymptomatic and flexible nasendoscopy demonstrated a small benign-appearing lesion on the right laryngeal aspect of the epiglottis. MRI identified a thickened epiglottis, with calcification and low T2 signal intensity. Panendoscopy revealed a firm lesion involving the lower half of the laryngeal surface of the epiglottis, particularly on the right side, crossing the midline and extending to the petiole. The remainder of the larynx was not involved. Histopathological examination of biopsies taken showed mucosa in which the lamina propria is largely replaced by amyloid. The patient was discussed at the head and neck multi-disciplinary meeting with the plan of ongoing observation. Her condition remained stable 4 months following her diagnosis. Discussion: Primary amyloidosis of the larynx is extremely rare and presents with non-specific symptoms and radiological findings. Tissue biopsy is therefore vital for diagnosis with histopathological findings being characterised by homogenous eosinophilic extra-cellular deposits which stain with Congo red under microscopy. Secondary causes of amyloidosis such as rheumatoid arthritis and myeloma may need to be excluded based on clinical suspicion. Treatment of primary head and neck amyloidosis is dependent on patient symptoms, with long term follow up recommended to monitor disease progression.
Conclusion: Isolated primary amyloidosis of the epiglottis is an extremely rare benign condition and diagnosis requires clinical suspicion in patients presenting with non-specific symptoms and benign-appearing laryngeal lesions. Tissue biopsy with histopathological examination is vital for diagnosis.