Recent Advances, Systemic Therapy, and Molecular Targets in Adenoid Cystic Carcinoma of the Head and Neck

With an incidence of 3-4.5 cases per million, adenoid cystic carcinoma (ACC) of the head and neck is one of the most common tumors of the parotid and sublingual salivary glands. In the clinical course, ACC is shown to have an aggressive long-term behavior, which leads to the fact that radical surgical resection of the tumor with tumor-free margins remains the gold standard in treating ACC. Particle radiation therapy and systemic molecular biological approaches offer new treatment options. However, risk factors for the formation and prognosis of ACC have not yet been clearly identified. The aim of the present review was to investigate long-term experience of diagnosis and treatment as well as risk and prognostic factors for occurrence and outcome of ACC.

Automated summary

With an incidence of 3-4.5 cases per million, adenoid cystic carcinoma (ACC) of the head and neck is a common tumor of the parotid and sublingual salivary glands. Radical surgical resection with tumor-free margins remains the gold standard in treating ACC, but there are also new treatment options available such as particle radiation therapy and systemic molecular biological approaches. The review aims to investigate the long-term experience of diagnosis and treatment, as well as risk and prognostic factors for ACC.