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Adenoid Cystic Carcinoma (ACC)

Adenoid cystic carcinoma (ACC) facts

Adenoid cystic carcinoma typically affects the salivary glands.
Adenoid cystic carcinoma typically affects the salivary glands.

*Adenoid cystic carcinoma (ACC) facts written by Charles P. Davis, MD, PhD

  • Adenoid cystic carcinoma (ACC) is a rare form of cancer (adenocarcinoma) that most often arises in the salivary glands but may occur in other locations like the breast or uterus.
  • Symptoms and signs vary. They may cause
    • painless masses in the mouth or face,
    • bulging eye or vision change,
    • changes in speech, and/or
    • respiratory problems.
  • Advanced tumors may be painful and cause nerve paralysis and/or facial numbness.
  • Doctors make a diagnosis with histologic evaluation from a biopsy of a tumor mass. There are no serum markers for the disease. Scans like CT, MRI, or PET may help determine the size and location of tumors.
  • Treatment may include surgery (the first choice of therapy), radiation (neutron beam may be most effective), and/or chemotherapy. The prognosis is fair to poor with this aggressive cancer.
  • The underlying cause of ACC is unknown but may develop from non-inherited genetic changes. There is no link between tobacco, alcohol, or HPV infection and ACC.
  • There is speculation that ACC may be associated with certain family cancer syndromes, but that remains to be determined. Five-year survival is about 89% and 15-year survival is about 40%. Recurrence after treatment is common. However, phase 2 trials of axitinib (Inlyta) showed extended PFS (progression-free survival) in a 2020 study.

Summary

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.

Cause

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.

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Inheritance

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.

Research

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.

Organizations

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.

Organizations Supporting this Disease

Adenoid Cystic Carcinoma Organization International
P.O. Box 15482
San Diego, CA 92175-5482
E-mail: [email protected]
Website: http://www.accoi.org/

Adenoid Cystic Carcinoma Research Foundation
P.O. Box 442
Needham, MA 02494
Telephone: (781) 248-9699
E-mail: http://www.accrf.org/html/contactus.php
Website: http://www.accrf.org/

The Oral Cancer Foundation
3419 Via Lido #205
Newport Beach, CA 92663
Telephone: 949-723-4400
E-mail: [email protected]
Website: http://oralcancerfoundation.org

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Reviewed on 6/29/2020
References
SOURCE:

Unites States. National Institutes of Health. Genetic and Rare Diseases Information Center. "Adenoid Cystic Carcinoma." June 21, 2017. <https://rarediseases.info.nih.gov/diseases/5743/adenoid-cystic-carcinoma>.
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