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

J Neurooncol. 2020 Dec;150(3):419-427. doi: 10.1007/s11060-019-03366-x. Epub 2020 Jan 2.


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.

Keywords: Adenoid cystic carcinoma; Anterior skull base; Central skull base; Head and neck cancer; Lateral skull base; Radiotherapy; Skull base surgery.

Publication types

  • Review

MeSH terms

  • Animals
  • Carcinoma, Adenoid Cystic / pathology
  • Carcinoma, Adenoid Cystic / therapy*
  • Combined Modality Therapy
  • Disease Management
  • Humans
  • Skull Base Neoplasms / pathology
  • Skull Base Neoplasms / therapy*