What is ACC?

ACC (Adenoid Cystic Carcinoma) is a rare and unique form of cancer that is known to be unpredictable in nature, with a typical growth pattern of being slow and gradual, but over time can be progressive, insidious and relentless. There are some general tendencies, such as the propensity for it to spread into surrounding nerve tissue or metastasize to other areas of the body, yet each patient can experience their own diverse patterns and issues. ACC occurs most commonly in the oral cavity with 58% of the primary tumors beginning in that area, but it can actually occur in as many as 38 different organs in the body. It is commonly considered to be a salivary gland tumor and grouped with other oral cancers in statistical studies since it occurs predominantly in that area. The oral area includes the major and minor salivary glands, roof of mouth (palate), floor of mouth, gums, tongue, pharynx and lips. Though it is often considered to be a salivary gland tumor, ACC actually occurs in the broader grouping of all types of secretory glands (glands that secrete fluids) including tear glands, sweat glands, mucus and excretory glands. Besides the oral cavity, ACC also occurs in the nose, nasal cavity, sinus, larynx, trachea, esophagus, ears, lungs, bronchus, brain, skin, lacrimal gland, breast, Bartholin's gland, vulva, cervix, and others. Upon initial diagnosis it is most often a single tumor that is located within a primary organ and in about 5% to 10% of the cases can include spread into lymph nodes. In a limited number of cases it may have already spread into adjoining areas through nerve invasion or metastasized to the lungs, liver or bone as well. This takes place most often when it has been misdiagnosed for year.

How Rare is ACC?

Of the several hundred thousand new cases of cancer diagnosed each year in the US, only about 1200 of them are ACC. Long term statistical information from the National Cancer Institute estimates that there are approximately 14,000 Americans alive today who are living with ACC or have dealt with it in the past. This is why it is classified as a rare or “orphan” disease. Many physicians see very few, if any, ACC patients in their practice since ACC occurs in only about one out of every 500 cases of cancer. This is the primary reason many patients have reported their difficulty in locating a physician with knowledge and experience with ACC. This is also why historically there has been very little funding provided for ACC research by the government, drug companies and large nonprofit cancer organizations. That is beginning to change.