Amyloidosis in the head and neck: CT findings with clinicopathological correlation

Purpose: To characterize the CT imaging features of head and neck amyloidosis and correlate with extent of disease and clinical outcomes. Materials and methods: This retrospective study included 80 patients with head and neck amyloidosis who underwent soft tissue neck CT imaging between November 2003 and April 2018. The CT imaging features including lesion distribution, morphology (focal, diffuse/circumferential, or combined), presence and pattern of calcification, (punctate or diffuse), and thickness of airway lesion were evaluated and compared with the extent of amyloidosis (localized or systemic), and clinical course (stable, no recurrence, or progression requiring repeated surgical treatment). Results: Localized disease (83.8%, 67/80) was most common with AL type (97.6%, 41/42) representing nearly all cases of head and neck amyloidosis. The larynx was the most frequently affected organ (60.0%, 48/80), specifically the glottis (43.8%, 35/80). Calcification was seen in 65.0% of cases (52/80). Non-airway or tongue lesions were significantly associated with systemic (92.3%, 12/13) as opposed to localized amyloidosis (4.5%, 3/67; P < 0.001). Repeated surgical treatment was significantly associated with laryngeal amyloidosis (35.3%, 12/34; P = 0.002) and multi-centric disease (33.3%, 10/30; P = 0.048). Airway wall thickness in patients who required repeated surgical treatment was significantly greater than in patients with stable or no recurrence disease (P = 0.016). Conclusion: Knowledge of the imaging features of head and neck amyloidosis can aid the diagnosis, disease monitoring, and prediction of patients requiring repeated surgical intervention.