What is the definition of Adenoid Cystic Carcinoma?
Adenoid cystic carcinoma (ACC) is a rare form of adenocarcinoma, a type of cancer that begins in glandular tissues. It most commonly arises in the major and minor salivary glands of the head and neck. It can also occur in the breast, uterus, or other locations in the body. Symptoms depend on the tumor's location. Salivary gland tumors may cause painless masses in the mouth or face. Tumors of the lacrimal gland may cause a bulging eye or changes in vision. Those affecting the windpipe or voice box may cause respiratory symptoms or changes in speech, respectively. Advanced tumors may cause pain and/or nerve paralysis, as ACC often spreads along the nerves. It may also spread through the bloodstream. It spreads to the lymph nodes in about 5% to 10% of cases. The cause of ACC is currently unknown. It typically does not run in families. Treatment depends on many factors and may include surgery, radiation, and/or chemotherapy. Unfortunately, ACC is typically an aggressive form of cancer that has a poor long-term outlook.
What are the alternative names for Adenoid Cystic Carcinoma?
- Adenocystic carcinoma
- Cribriform carcinoma
What are the causes for Adenoid Cystic Carcinoma?
The underlying cause of adenoid cystic carcinoma (ACC) is not yet known, but it appears to develop from non-inherited, genetic changes that occur during a person's lifetime. These genetic changes are present only in the cancer cells, not in the cells with the genetic material that is passed on to offspring (the egg and sperm cells). The changes may be caused by exposures in a person's environment. However, no strong environmental risk factors specific to ACC have been identified. 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 (HPV).
There has been evidence that ACC tumor cells are associated with the presence of too much of a protein called “myb,” and with the p53 tumor suppressor gene. This gene normally limits cell growth by monitoring the rate at which cells divide. Research is under way to better understand how ACC develops.
Is Adenoid Cystic Carcinoma an inherited disorder?
Adenoid cystic carcinoma (ACC) is not considered to be an inherited cancer. Like most cancers, ACC appears to develop from genetic changes that are caused by something in a person's environment. In almost all reported cases, ACC has occurred sporadically in people with no family history of ACC. However, we are aware of two reports in the literature in which more than one family member had ACC. There has also been speculation about whether ACC may be associated with certain family cancer syndromes, but more research is needed to determine if an association exists.