Adenoid Cystic Carcinoma: Introduction

Approved by the Cancer.Net Editorial Board, 08/2020

ON THIS PAGE: You will find some basic information about this disease and the parts of the body it may affect. This is the first page of Cancer.Net’s Guide to Adenoid Cystic Carcinoma. Use the menu to see other pages. Think of that menu as a roadmap for this complete guide.

Cancer begins when healthy cells change and grow out of control, forming a mass called a tumor. A tumor can be cancerous or benign. A cancerous tumor is malignant, meaning it can grow and spread to other parts of the body. A benign tumor means the tumor can grow but will not spread.

About adenoid cystic carcinoma

Adenoid cystic carcinoma (AdCC) is a rare form of adenocarcinoma, which is a broad term describing any cancer that begins in glandular tissues. 

In general, AdCC is found mainly in the head and neck region. It can occasionally occur in other locations in the body, including the breasts or a woman’s uterus. AdCC most commonly occurs in the salivary glands, which consist of clusters of cells that secrete saliva scattered throughout the upper aerodigestive tract. The upper aerodigestive tract includes the organs and tissues of the upper respiratory tract, such as the lips, mouth, tongue, nose, throat, vocal cords, and part of the esophagus and windpipe. Salivary glands are generally divided into 2 groups based on their size: minor salivary glands and major salivary glands. A tumor may begin in the following places in these 2 groups:

  • Minor salivary glands

    • Palate — roof of the mouth

    • Nasopharynx — an 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 — the inner lining of the mouth; glands located here produce mucus

    • Larynx — the voice box

    • Trachea — the windpipe

  • Major salivary glands

    • Parotid glands — the largest salivary gland found on either side of the face in front of each ear

    • Submandibular glands — found under the jawbone

    • Sublingual glands — located in the bottom of the mouth under the tongue

Regardless of where it starts, AdCC tends to spread along nerves, known as a perineural invasion, or through the bloodstream. It spreads to the lymph nodes in about 5% to 10% of cases. If it spreads to another part of the body beyond the lymph nodes, it is called metastatic cancer. The most common place of AdCC metastases is the lung. AdCC is known for having long periods of no growth, or indolence, followed by growth spurts. But AdCC can behave aggressively in some people, making the course of the AdCC unpredictable.

Besides being classified based on where the cancer begins, AdCC is also described based on what the tumor cells look like under a microscope. This is called the histologic variations of the tumor. The tumor can be classified as:

  • cylindroma, a tumor with tube-shaped cells

  • cribriform, a tumor with gaps between the cells, giving it the appearance of Swiss cheese

  • solid AdCC

AdCC is sometimes classified as a disease of the minor salivary gland, even though it may begin at other locations. 

This guide covers AdCC. Learn more about other types of salivary gland cancers and other head and neck cancers in separate guides on this website.

The next section in this guide is StatisticsIt helps explain the number of people who are diagnosed with AdCC and general survival rates. Use the menu to choose a different section to read in this guide.