Overview

Adenoid cystic carcinoma (AdCC) is a rare form of adenocarcinoma, which is a broad term describing any cancer arising from glandular tissues. AdCC is found mainly in the head and neck, but can occasionally occur in a woman’s uterus or other sites in the body. It most commonly occurs in the salivary glands, which consist of clusters of secretory cells scattered throughout the upper aerodigestive tract (the organs and tissues of the upper respiratory tract, including the lips, mouth, tongue, nose, throat, vocal cords, and part of the esophagus and windpipe). Therefore, a tumor may begin in such areas as the:

• palate (roof of the mouth)
  
  
• nasopharynx (air passageway at the upper part of the throat and behind the nose)
  
  
• tongue base (the back third of the tongue)
  
  
• mucosal lining of the mouth (inner lining of the mouth; glands located here produce mucus)
  
  
• larynx (voicebox)
  
  
• trachea (windpipe)
  
  
• major salivary glands (the parotid, submandibular, or sublingual glands).

Regardless of where it starts, AdCC has the same basic biologic behavior. AdCC tends to spread along nerves (perineural invasion) or through the bloodstream. It only spreads to the lymph nodes in about 5% to 10% of cases. The most common place of metastases (spreading) is the lung. AdCC is known for having long periods of indolence (no growth), followed by growth spurts. However, AdCC can behave aggressively in some people, making the course of the AdCC unpredictable.

There are many other terms that are used to describe AdCC, which mostly refer to histologic (how cells look under a microscope) variations of the tumor, including cylindroma, cribiform, and solid AdCC. AdCC is sometimes classified as a disease of the minor salivary gland, even though it may begin at other sites.

For more information on head and neck cancers, read Cancer.Net’s Guide to Head and Neck Cancer.

Statistics

AdCC is most often diagnosed in people in their 40s to 60s, but there are documented cases of pediatric (childhood) AdCC. Women are slightly more likely to be diagnosed with AdCC than men (the female to male ratio is approximately 3 to 2).

The five-year relative survival rate (the percentage of people who survive at least five years after the cancer is detected, excluding those who die from other diseases) of people with AdCC is approximately 89%. The 15-year relative survival rate (the percentage of people who survive at least 15 years after the cancer is detected, excluding those who die from other diseases) of people with AdCC is approximately 40%. Tumor growth for AdCC is often slow, and people may live a long time with metastatic disease; however, a late recurrence (cancer that comes back after treatment) of AdCC is common and can occur many years after initial treatment.

Cancer survival statistics should be interpreted with caution. These estimates are based on data from thousands of cases of this type of cancer in the United States each year, but the actual risk for a particular individual may differ. It is not possible to tell a person how long he or she will live with AdCC. Because survival statistics are often measured in multi-year intervals, they may not represent advances made in the treatment or diagnosis of this cancer

Source: University of Virginia Health System.

Find out more about basic cancer terms used in this section.