Management of advanced adenoid cystic carcinoma infiltrating the skull base: a contemporary review

Introduction This article provides an overview of the natural history and management of adenoid cystic carcinoma infiltrating the skull base (SB ACC). Methods A comprehensive review of the available literature and discussion regarding the current management of SB ACC. Results This review describes the unique pathology, appropriate diagnostic work-up and contemporary management options in SB ACC. There is a specific focus on the role of surgical resection with post-operative radiotherapy, given the literature suggesting a superior locoregional control rate. The importance of surgical decision making and varying surgical approaches are detailed. In addition, the emerging role of biological agents is discussed. Conclusion The gold standard for SB ACC is surgical resection and post-operative radiation, given the superior locoregional control. In patients not amendable to surgery, palliative radiation should be considered. The role of biological agents is still evolving. The complexity of SB ACC management mandates experienced multi-speciality management.