Chemotherapy and targeted therapy in adenoid cystic carcinoma of the head and neck: a review.

Adenoid cystic carcinoma (ACC) is an uncommon tumor usually arising in the head and neck region, mainly in the salivary glands. It demonstrates an indolent prolonged course and is characterized by perineural invasion. Primary treatment of local and locoregional disease consists mainly of surgery and/or irradiation. During follow-up these patients frequently develop local recurrences and distant metastases, especially in the lung, although long-term survival is possible. The role of chemotherapy in ACC is limited, and studies with only a limited number of patients are performed. In this article we review the literature on chemotherapy regimens, including monotherapy and combination chemotherapy schedules, as well as the new targeted therapies.