What is Adenoid Cystic Carcinoma?
Adenoid cystic carcinoma (ACC) is an uncommon form of malignant neoplasm that arises within secretory glands, most commonly the major and minor salivary glands of the head and neck. Other sites of origin include the trachea, lacrimal gland, breast, skin, and vulva. This neoplasm is defined by its distinctive histologic appearance.
Adenoid Cystic Carcinoma (ACC) is a rare malignancy of the head and neck, 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 uterus. There are approximately 600 new cases of ACC diagnosed each year in the United States with a median age at diagnosis of 43. This cancer has a slow, and sometimes relentless, progression characterized by frequent recurrences and metastases to sites such as the lungs, liver and bones. This peculiar progression leads to relatively high 5-year cancer survival rates, but less optimistic outcomes as time from diagnosis proceeds. While infiltration to the lymph nodes is not common, ACC is one of the very few cancers that infiltrate and spread along nerves (perineural invasion). ACC has no known causes or links to smoking and alcohol consumption. The disease is slightly more prevalent in women than men. Standard treatment for ACC in
