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What is Adenoid Cystic Carcinoma?
Cancer begins when normal cells change and grow uncontrollably, forming a mass called a tumor. A tumor can be benign (noncancerous) or malignant (cancerous, meaning it can spread to other parts of the body).
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 it 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 cells that secrete saliva scattered throughout the upper aerodig estive 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). A tumor may begin in the:
Minor salivary glands:
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)
Major salivary glands:
Parotid (largest salivary gland found on either side of the face in front of the ears)
Submandibular (found under the jawbone)
Sublingual glands (in the bottom of the mouth under the tongue)
Regardless of where it starts, AdCC tends to spread along nerves (perineural invasion) or through the bloodstream. It spreads to the lymph nodes in only 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.
Besides being classified based on where the cancer begins, AdCC is also described based on the 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.
Symptoms and Signs
People with AdCC may experience the following symptoms or signs. Sometimes, people with AdCC do not show any of these symptoms. Or, these symptoms may be caused by a medical condition that is not cancer. If you are concerned about a symptom or sign on this list, please talk with your doctor.
The initial symptoms of AdCC depend on the location of the tumor. Early lesions of the salivary glands may appear as painless, usually slow-growing masses underneath the normal lining of the mouth or skin of the face. Because there are many salivary glands under the mucosal lining of the mouth, throat, and sinuses, lumps in these locations could be from this type of tumor. Other symptoms may include:
A lump on the palate, under the tongue, or in the bottom of the mouth
An abnormal area on the lining of the mouth
Numbness of the upper jaw, palate, face, or tongue
A bump or nodule in front of the ear or underneath the jaw
Paralysis of a facial nerve
Your doctor will ask you questions about the symptoms you are experiencing to help find out the cause of the problem, called a diagnosis. This may include how long you’ve been experiencing the symptom(s) and how often.
If cancer is diagnosed, relieving symptoms and side effects remains an important part of cancer care and treatment. This may also be called symptom management, palliative care, or supportive care. Be sure to talk with your health care team about symptoms you experience, including any new symptoms or a change in symptoms.
A risk factor is anything that increases a person’s chance of developing cancer. Although risk factors often influence the development of cancer, most do not directly cause cancer. Some people with several risk factors never develop cancer, while others with no known risk factors do. However, knowing your risk factors and talking about them with your doctor may help you make more informed lifestyle and health care choices.
The cause of AdCC is unknown at this time, and risk factors for this type of cancer have not been proven consistently with scientific research. There is some evidence that the p53 tumor suppressor gene (a gene that limits cell growth by monitoring the rate at which cells divide) is somehow inactivated in advanced and aggressive forms of AdCC.
Doctors use many tests to diagnose cancer and find out if it has metastasized (spread). Some tests may also determine which treatments may be the most effective. For most types of cancer, a biopsy is the only way to make a definitive diagnosis of cancer. If a biopsy is not possible, the doctor may suggest other tests that will help make a diagnosis. Imaging tests may be used to find out whether the cancer has metastasized. Your doctor may consider these factors when choosing a diagnostic test:
Age and medical condition
Type of cancer suspected
Severity of symptoms
Previous test results
In addition to a physical examination, the following tests may be used to diagnose AdCC:
Biopsy. A biopsy is the removal of a small amount of tissue for examination under a microscope. Other tests can suggest that cancer is present, but only a biopsy can make a definite diagnosis. The sample removed from a biopsy is analyzed by a pathologist (a doctor who specializes in interpreting laboratory tests and evaluating cells, tissues, and organs to diagnose disease). The pathology of the salivary gland may be complicated even among experienced pathologists so it is important that the tissue is examined by a head and neck pathologist who is experienced in diagnosing salivary disease.
The biopsy can be performed by using a fine needle biopsy or by surgical removal of part or all of the tumor. A fine needle biopsy is also called fine needle aspiration or FNA. This procedure uses a thin needle to remove fluid and cells. An AdCC tumor is characterized by a distinctive pattern in which bundles of epithelial cells surround and/or infiltrate ducts or glandular structures within the organ. Frequently, diagnosis of AdCC is made after the surgical removal of a tumor first thought to be benign.
Imaging tests. Imaging techniques, primarily magnetic resonance imaging (MRI) or computed tomography (CT) scan, are useful to help doctors see the extent of the tumor before any surgery. A positron emission tomography (PET) scan may also be used to determine if the tumor has spread to other parts of the body.
An MRI uses magnetic fields, not x-rays, to produce detailed images of the body. A contrast medium (a special dye) may be injected into a patient’s vein to create a clearer picture. An MRI is very useful for identifying perineural spread (growth of the tumor along nerve branches) of AdCC.
A CT scan creates a three-dimensional picture of the inside of the body with an x-ray machine. A computer then combines these images into a detailed, cross-sectional view that shows any abnormalities or tumors. Sometimes, a contrast medium is injected into a patient’s vein to provide better detail.
A PET scan is a way to create pictures of organs and tissues inside the body. A small amount of a radioactive substance is injected into a patient’s body. This substance is absorbed mainly by organs and tissues that use the most energy. Because cancer tends to use energy actively, it absorbs more of the radioactive substance. A scanner then detects this substance to produce images of the inside of the body.