Curative approaches for adenoid cystic carcinoma: a multifaceted disease

Adenoid cystic carcinoma (AdCC) of the salivary glands is a rare malignancy characterized by a poor outcome at long term follow-up. While the locoregional treatment of the primary tumor is mainly based on surgery and radiation therapy, the disease typically recurs at distance, for which curative options are frequently lacking. Despite advancements in therapeutic approaches, to date the optimal management strategy in the recurrent/metastatic setting remains unclear due to the limited availability of high-quality evidence, thus optimization of curative treatment is of outmost importance. This narrative review explores the current state of curative treatments, and their role in achieving locoregional control and disease-free survival. Furthermore, clinicopathological and molecular biological key prognostic factors are analyzed to provide insights into association with treatment outcome. This review also highlights the need for multidisciplinary treatment planning and identifies gaps in the literature, emphasizing areas requiring further research to optimize patient care.