Adenoid Cystic Carcinoma (ACC) is a rare malignancy of secretory glands, typically originating in the salivary glands though it does appear in other primary sites, including the palate, nasopharynx, tongue base, larynx, trachea, lacrimal gland, breast and vulva. There are approximately 1,200 new cases of ACC diagnosed each year in the United States, about 60% of which involve women. This cancer has a slow, and sometimes relentless, progression characterized by frequent recurrences and metastases to sites such as the lungs, liver and bones.
Cancer researchers point to genetic alterations as the underlying cause of all cancers. Only a small portion (perhaps 10%) of cancers is believed to be inherited, and ACC is not one of these inheritable cancers. Rather, as with the vast majority of cancers, ACC appears to develop from genetic alterations caused by a person´s environment, such as through exposure to radiation or carcinogens. Unlike some other cancers of the head and neck, ACC is not linked to tobacco or alcohol use or infection by the human papilloma virus.
Although the definitive cause of ACC is not known at the moment, there is very strong evidence that ACC tumor cells are driven by the presence of too much of a protein called “myb”. ACCRF-affiliated researchers are undertaking extensive studies to understand how to interfere with the molecular pathways that permit “myb” to spur tumor growth. Undoubtedly, better understanding of the biology of ACC will lead to better treatments for patients.