Head and neck adenoid cystic carcinoma: What is new in biological markers and treatment?

Purpose of Review: The purpose of this article is to provide a current review of publications on the molecular and basic science research findings for adenoid cystic carcinoma. Recent Findings: Findings related to adenoid cystic carcinoma carcinogenesis are discussed, including those involving cytogenetics, oncogenes, epigenetic alterations, biomarker studies, xenografts, and cancer stem-like cells. The defining molecular feature of adenoid cystic carcinoma is the presence of a recurrent chromosomal translocation, t(6; 9) (q22-23; p23-24), with the fusion transcript involving the genes MYB and NFIB. Molecular markers have been investigated and different targeted therapies have been explored, but unfortunately, none of these efforts has, to date, significantly improved outcomes in these patients. Summary: The identification of molecular abnormalities underlying adenoid cystic carcinoma and those responsible for carcinogenesis is paramount to the development of specific targeted therapies. The design of robust clinical trials with embedded translational research is critical in determining the dosing, schedules, and combinations of such therapies. Further progress in this challenging field will require multicenter cooperation to compile molecular databases and to initiate prospective trials to determine the roles of promising new agents.