Adenoid cystic carcinoma (ACC) of the salivary glands is the second most common type of salivary gland cancer, and is characterized by a poor prognosis and an unclear pathology. The incidence of ACC is rare, as it accounts for 10‑15% of all salivary gland tumors and affects mainly patients aged between 50 and 60 years. The annual incidence rate is estimated to be ~4.5 cases per 100,000 individuals. Due to its rarity and the use of contaminated cell lines in previous investigations, the precise etiological factors underlying ACC remain poorly understood. Current treatment modalities, typically involving surgery with or without postoperative radiotherapy, often prove unsatisfactory due to the potential for local recurrence and delayed distant metastases, which may manifest 3‑5 years after treatment and constitute the primary failure of existing therapeutic approaches. The indolent growth pattern, along with perineural and perivascular invasion, is potentially responsible for the delayed onset of metastases. No effective systemic therapy has been established so far. Therefore, the management of ACC represents a significant therapeutic challenge. Exploring the molecular characteristics of ACC, including the reasons behind its propensity for perineural invasion and its potential correlation with the immune system, offers promising strategies for managing ACC and could open up novel pathways for future therapeutic interventions. Currently, the use of immunotherapy in ACC treatment has shown limited effectiveness. While the exact mechanism underlying the lack of response to immunotherapy in ACC remains unknown, the low levels of tumor‑infiltrating lymphocytes in these tumors may contribute to this resistance. Therefore, identifying novel targets to enhance the immune response against tumor cells is essential. The present review provides an update on clinical studies and explores novel therapeutic targets that could be effective in the therapeutic management of ACC.

Related Articles