PURPOSE OF REVIEW

Adenoid cystic carcinoma (ACC) is a rare cancer of the secretory glands, typically originating in the salivary glands of the head and neck. The impact of chemotherapy on survival is unclear and there are no standard-of-care treatments for patients with recurrent or metastatic disease. This article reviews recently completed and ongoing clinical trials for patients with ACC and describes recently identified potentially targetable genomic alterations in this orphan disease.

RECENT FINDINGS

In spite of an overall low mutational burden, genotyping of ACC samples has shed some light about the disease biology. In addition to the frequent translocations involving MYB or MYBL, recurrent alterations in genes involved in chromatin deregulation, FGF, PI3K, NOTCH1, and DNA damage repair pathways have been identified. Many of these genomic alterations are targetable and drug screening is ongoing in genotyped ACC patient-derived murine xenografts.

SUMMARY

Clinical studies with targeted agents in unselected ACC patients have not been promising thus far. The identification of potential driver oncogenes suggests that targeted therapy might be effective in molecularly-defined patient subgroups and merits investigation in future clinical studies.