Adenoid cystic carcinoma of the larynx: a 40-year experience.

BACKGROUND Laryngeal adenoid cystic carcinoma (ACC) is a rare disease. We reviewed our experience at the Princess Margaret Hospital (PMH) with its treatment. METHODS This is a retrospective chart review of 15 cases treated at PMH between 1963 and 2005. RESULTS The mean age was 48.6 years. There was no sex predilection. The subglottis was the most common subsite involved. Only 2 patients had regional metastasis. Local or regional recurrence was noted in 5 patients (33.3%). The distant metastasis rate was 66.7% and involved the lungs. The median follow-up time was 6.9 years. The 5- and 10-year overall and disease-specific survival rates were 64% and 46%, and 69% and 49%, respectively. CONCLUSION Laryngeal ACC is a rare disease with a high rate of distant recurrence. Its management should emphasize maximizing local and regional disease control by surgery followed by radiotherapy with distant disease failure eventually dictating survival.