Abstract
PURPOSE: Adenoid cystic carcinoma (ACC) of the trachea is a rare malignancy. We report a patient with ACC who underwent multimodal management including tracheal resection. A systematic review was also conducted on tracheal resection for ACC. METHODS: A literature search was conducted on MEDLINE, Embase, and PubMed using the search terms "trachea AND adenoid cystic carcinoma AND (surgery OR resection)" and articles from 2000 to August 2021 were identified. A total of 29 journal articles were included in the review. RESULTS: A total of 403 patients underwent surgery for tracheal ACCs. The mean age was 48.1 years and 54.7% were female. The commonest anatomical location was the lower trachea (46.9%). The mean time from symptom onset to diagnosis was 16.6 months with the commonest symptom being dyspnoea (52%). Fifty-eight percent of the patients had intraluminal growth. Tracheal resection (46.2%) and access via thoracotomy (41.4%) were the commonest procedures described. The mean length of trachea resected was 39.2 mm and the mean tumour size was 31.5 mm. 16.8% of lymph nodes were involved and 73.8% of cases had positive resection margins. The overall complication rate was 1.4-5.4% and the in-hospital mortality rate was 9.8%. The overall survival reported was 61.7% at 5 years and 54.6% at 10 years. CONCLUSION: Surgical resection followed by adjuvant radiotherapy is the mainstay in the treatment of tracheal ACC, notwithstanding the high rates of involved margins. Achieving tension-free anastomosis should be the first priority given the favourable response of adjuvant therapies in reducing recurrence rate and improving overall survival.