Clinical Features, Treatment, and Outcomes for Oropharyngeal Adenoid Cystic Carcinoma: A Systematic Review

OBJECTIVE: To describe the presenting symptoms, treatment regimens, and outcomes associated with oropharyngeal adenoid cystic carcinoma. DATA SOURCES: Ovid MEDLINE(R), Ovid Embase, Ovid Cochrane Central Register of Controlled Trials, and Scopus via Elsevier. REVIEW METHODS: This review followed the Preferred Reporting Items for Systematic Reviews and Meta-analyses. A medical librarian conducted the literature search using controlled vocabulary and keywords, with investigator input. Eligible articles were clinical trials and observational studies reporting on clinical features, treatment, or outcomes for oropharyngeal adenoid cystic carcinoma. RESULTS: Our search identified 233 unique citations, with 44 articles included covering 195 patients. The female-to-male ratio was 1.7:1, and the base of the tongue was the most common subsite (73%). Presenting symptoms were mainly pain/discomfort (n = 31), followed by a palpable mass (n = 19) and dysphagia (n = 12). At diagnosis, 22.3% (29 patients) had positive lymph nodes. Treatment was primarily surgical (n = 102), with definitive radiation therapy used in 58 cases. Disease progression was noted in 86 patients, 36% (n = 31) of whom developed distant metastases-primarily to the lungs (n = 26). Long-term outcomes (n = 76) showed 3- and 5-year overall survival rates of 71.5% to 100% and 47.6% to 79.0%, and disease-free survival rates of 54.7% to 70.0% and 21.9% to 44.0%, respectively. CONCLUSION: Among patients with available data, oropharyngeal adenoid cystic carcinoma appeared more common in women and has a predilection for the base of the tongue. However, this finding should be interpreted with caution, given the high proportion of missing data. These tumors appear to carry a higher risk for nodal metastases and may lead to poor outcomes.