Management of advanced adenoid cystic carcinoma infiltrating the skull base: a contemporary review
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TOPIC REVIEW
Management of advanced adenoid cystic carcinoma infiltrating the skull base: a contemporary review E. Guazzo1,2 · B. Panizza1,2,3 Received: 2 November 2019 / Accepted: 9 December 2019 © Springer Science+Business Media, LLC, part of Springer Nature 2020
Abstract 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 · Skull base surgery · Anterior skull base · Lateral skull base · Central skull base · Head and neck cancer · Radiotherapy
Introduction Adenoid cystic carcinoma (ACC) is a primary salivary neoplasm with a unique biological behaviour resulting in propensity for skull base invasion [1, 2]. The treatment consensus in ACC is resection to negative surgical margins followed by post-operative radiation (PORT) [2–7]. However, in skull base disease, oncological resection is uniquely challenging often requiring a multi-speciality ablative and reconstructive approach [8–10]. In this contemporary review, we discuss the pathology and natural history of skull base ACC (SB ACC), current investigative and management options,
* B. Panizza [email protected] 1
Queensland Skull Base Unit, Department of Otolaryngology, Head and Neck Surgery, Princess Alexandra Hospital, Brisbane, Australia
2
Faculty of Medicine, University of Queensland, Brisbane, Australia
3
Department of Neurosciences, Mater Hospital of Brisbane, South Brisbane, Australia
surgical approaches and oncological outcomes to assist in decision making in this unique skull base malignancy.
Natural history of ACC The incidence of ACC is estimated at approximately 3–4.5 cases per million, with skull base infiltration representing the more advanced tumours [2, 11]. The clinical behaviour of ACC is best described as paradoxical, with slow tumour growth but diffuse advancement of disease at the primary site, and high rates of locoregional recurrence and metastatic disease to both lung and bone. Regional lymph node metastases are regarded as rare [2, 3, 12–14]. Literature examining the natural history a
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