National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences

Adenoid cystic carcinoma


Other Names:
Adenocystic carcinoma; Cribriform carcinoma; Cylindroma
Categories:
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.[1] It can also occur in the breast, uterus, or other locations in the body.[1][2] 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.[2] Advanced tumors may cause pain and/or nerve paralysis, as ACC often spreads along the nerves.[1][2] It may also spread through the bloodstream. It spreads to the lymph nodes in about 5% to 10% of cases.[1] 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.[1][2] Unfortunately, ACC is typically an aggressive form of cancer that has a poor long-term outlook.[3]

Last updated: 6/21/2017
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).[2] The changes may be caused by exposures in a person's environment. However, no strong environmental risk factors specific to ACC have been identified.[2] 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).[4]

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.[4][1] This gene normally limits cell growth by monitoring the rate at which cells divide.[1] Research is under way to better understand how ACC develops.[4]
Last updated: 6/21/2017
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.[4] 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.[5][6] 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.[7][8]
Last updated: 6/21/2017

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

  • ClinicalTrials.gov lists trials that are related to Adenoid cystic carcinoma. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

    Please note: Studies listed on the ClinicalTrials.gov website are listed for informational purposes only; being listed does not reflect an endorsement by GARD or the NIH. We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study.

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

  • The Oral Cancer Foundation provides information about adenoid cystic carcinoma. Visit the above link to access this information.
  • Cancer.Net, a resource from the American Society of Clinical Oncology, provides information about adenoid cystic carcinoma. Click on the above link to access this information.
  • The National Cancer Institute provides the most current information on cancer for patients, health professionals, and the general public.
  • The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.

In-Depth Information

  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Adenoid cystic carcinoma. Click on the link to view a sample search on this topic.

Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know. Submit a new question

  • We would like to know if this type of cancer can be environmental. My father in law worked at an automotive company for over 30 years and he also lived very close to the airport. His house was right in the landing path of the airplanes. Sometimes the planes were so low that you could actually see the people in the windows of the plane. Also, is this cancer genetic? See answer


  1. Adenoid Cystic Carcinoma. Cancer.Net (American Society of Clinical Oncology). January, 2016; http://www.cancer.net/cancer-types/adenoid-cystic-carcinoma/overview.
  2. Adenoid Cystic Carcinoma. Oral Cancer Foundation. http://www.oralcancerfoundation.org/facts/rare/ac/. Accessed 6/20/2017.
  3. Mikse OR, Tchaicha JH, Akbay EA, Chen L, Bronson RT, Hammerman PS, Wong KK. The impact of the MYB-NFIB fusion proto-oncogene in vivo. Oncotarget. May 31, 2016; 7(22):31681-31688. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5077968/.
  4. Understanding ACC. Adenoid Cystic Carcinoma Research Foundation. https://www.accrf.org/living-with-acc/understanding-acc/. Accessed 6/21/2017.
  5. Yoshioka N, Mese H, Okui T, Ibaragi S, Sasaki A. Familial adenoid cystic carcinoma of sublingual salivary glands. Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology. May, 2015; 27(3):353-356. http://www.sciencedirect.com/science/article/pii/S2212555814000477.
  6. Channir HI, van Overeem Hansen T, Andreasen S, Yde CW, Kiss K, Charabi BW. Genetic Characterization of Adenoid Cystic Carcinoma of the Minor Salivary Glands: A Potential Familial Occurrence in First-Degree Relatives. Head Neck Pathol. February 16, 2017; [Epub ahead of print]:https://www.ncbi.nlm.nih.gov/pubmed/28210977.
  7. Shen TK, Teknos TN, Toland AE, Senter L, Nagy R. Salivary Gland Cancer in BRCA-Positive Families : A Retrospective Review. JAMA Otolaryngol Head Neck Surg. September 25, 2014; [Epub ahead of print]:Accessed 12/10/2014.
  8. Yilmaz B, Goldberg LH, Schechter NR, Kemp BL, Ruiz H. Basal cell nevus syndrome concurrent with adenoid cystic carcinoma of salivary gland. J Am Acad Dermatol. May, 2003; 48(5 Suppl):S64-66.