Laryngeal Adenoid Cystic Carcinoma: A Systematic Review

Objective: Adenoid cystic carcinoma is a malignant minor salivary gland tumor that represents <1% of all laryngeal tumors. The submucosal location of laryngeal adenoid cystic carcinoma (LACC) results in delayed presentation. Here, we present the first systematic review of reported cases of LACC to determine trends in presentation, diagnostic and treatment modalities, and patient outcome.

Data sources: PubMed, Web of Science, MEDLINE, and EMBASE databases.

Methods: A search of the above databases was done to identify articles reporting cases of LACC. The variables included in the analysis were patient demographics, presenting symptoms, tumor location, imaging, treatment, follow-up time, recurrence, and outcome.

Results: A total of 50 articles and 120 cases were included in the review. The most common presenting symptom was dyspnea (48.8%), followed by hoarseness (43.9%). LACC arose most frequently from the subglottis (56.7%). At presentation, 14.6% (13 of 89) of patients had regional disease. The average follow-up time was 54.0 months. At follow-up, distant metastasis was reported in 30 cases (33.3%). Surgery alone (43.3%) and surgery with radiotherapy (43.3%) were used most frequently and resulted in 57.1% and 55.3% of patients alive with no evidence disease at follow-up, respectively.

Conclusion: LACC was most often located in the subglottis. Patients commonly presented with dyspnea and hoarseness. In this systematic review, surgery with radiotherapy and surgery alone were the most commonly employed treatment modalities, and both resulted in slightly more than 50% of patients alive with no evidence of disease at follow-up.