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 patients 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 patients 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 patients 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 the survival statistics are measured in five-year (or sometimes one-year) intervals, they may not represent advances made in the treatment or diagnosis of this cancer.
A risk factor is anything that increases a person’s chance of developing cancer. Some risk factors can be controlled, such as smoking, and some cannot be controlled, such as age and family history. Although risk factors can 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 communicating them to your doctor may help you make more informed lifestyle and health-care choices.
The cause of AdCC is unknown at this time. 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.
People with AdCC may experience the following symptoms. 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 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 of the mouth or 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:
Difficulties in swallowing
Hoarseness
Dull pain
A bump or nodule in front of the ear or underneath the jaw
Doctors use many tests to diagnose cancer and determine 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
The 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). This procedure can be performed by using a fine needle biopsy, or by surgical removal of part or the entire tumor. A fine needle biopsy is also called fine needle aspiration or FNA. This procedure uses a thin needle to withdraw 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 (non-cancerous).
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 prior to 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 (growth of the tumor along nerve branches) spread 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 and absorbed by the organs or tissues being studied. This substance gives off energy that is detected by a scanner, which produces the images.
Staging is a way of describing a cancer, such as where it is located, if or where it has spread, and if it is affecting the functions of other organs in the body. Doctors use diagnostic tests to determine the cancer’s stage, so staging may not be complete until all of the tests are finished. Knowing the stage helps the doctor to decide what kind of treatment is best and can help predict a patient’s prognosis (chance of recovery). There are different stage descriptions for different types of cancer.
There is no uniform staging system used for AdCC, but often the staging system for a major salivary gland tumor is used.
One tool that doctors use to describe the stage is the TNM system. This system uses three criteria to judge the stage of the cancer: the size of the tumor itself, whether or not the tumor has spread to the lymph nodes around the tumor, and if the tumor has spread to other parts of the body. The results are combined to determine the stage of cancer for each person. There are five stages: stage 0 (zero) and stages I through IV (one through four). The stage provides a common way of describing the cancer so doctors can work together to plan the best treatments.
TNM is an abbreviation for tumor (T), node (N), and metastasis (M). Doctors look at these three factors to determine the stage of cancer:
How large is the primary tumor and where is it located? (Tumor, T)
Has the tumor spread to the lymph nodes? (Node, N)
Has the cancer metastasized to other parts of the body? (Metastasis, M)
Tumor. Using the TNM system, the "T" plus a letter or number (0 to 4) is used to describe the size and location of the tumor. Some stages are divided into smaller groups that help describe the tumor in even more detail. Specific tumor stage information is listed below.
TX: Indicates the primary tumor cannot be evaluated.
T0: No evidence of a tumor is found.
T1: Describes a small noninvasive tumor that is 2 centimeters (cm) at its greatest dimension.
T2: Describes a larger noninvasive tumor, between 2 cm to 4 cm.
T3: Describes a tumor that is larger than 4 cm, but not larger than 6 cm, that has spread beyond the salivary gland. However, the tumor does not affect the seventh nerve, which is the facial nerve that controls such expressions as smiles or frowns.
T4a: The tumor invades the skin, jawbone, ear canal, and/or facial nerve.
T4b: The tumor invades the skull base and/or the nearby bones and/or encases the arteries.
Node. The “N” in the TNM staging system is for lymph nodes, the tiny, bean-shaped organs that help fight infection. For AdCC, lymph nodes near the head and neck are called regional lymph nodes. Lymph nodes in other parts of the body are called distant lymph nodes.
NX: Indicates the regional lymph nodes cannot be evaluated.
N0: There is no evidence of cancer in the regional nodes.
N1: Indicates that cancer has spread to a single node on the same side as the primary tumor and the cancer found in the node is 3 cm or smaller.
N2: Describes any of these conditions:
N2a: Cancer has spread to a single lymph node on the same side as the primary tumor, and is larger than 3 cm, but not larger than 6 cm.
N2b: Cancer has spread to more than one lymph node on the same side as the primary tumor, and no tumor measures larger than 6 cm.
N2c: Cancer has spread to more than one lymph node on either side of the body, and no tumor measures larger than 6 cm.
N3: Cancer found in lymph nodes is larger than 6 cm.
Distant metastasis. The "M" in the TNM system describes cancer that has spread to other parts of the body.
MX: Indicates distant metastasis cannot be evaluated.
M0: Indicates the cancer has not spread to other parts of the body.
M1: Describes cancer that has spread to other parts of the body.
Cancer stage grouping
Doctors assign the stage of the cancer by combining the T, N, and M classifications.
Stage I: Describes a noninvasive tumor (T1, T2) with no spread to lymph nodes (N0) and no distant metastasis (M0).
Stage II: Describes an invasive tumor (T3) with no spread to lymph nodes (N0) or distant metastasis (M0).
Stage III: Describes a smaller tumor (T1, T2) that has spread to regional lymph nodes (N1), but have no sign of metastasis (M0).
Stage IVA: Describes any invasive tumor (T4a) with either no lymph node involvement (N0) or spread to only a single, same-sided lymph node (N1), but no metastasis (M0). It is also used for a T3 tumor with one-sided nodal involvement (N1), but no metastasis (M0), or any tumor (T) with extensive nodal involvement (N2).
Stage IVB: Describes any cancer (any T) with more extensive spread to lymph nodes (N2, N3) and no metastasis (M0).
Stage IVC: Describes any cancer (any T, any N) with distant metastasis (M1).
Recurrent: Recurrent cancer is cancer that comes back after treatment.
Used with permission of the American Joint Committee on Cancer (AJCC), Chicago, Illinois. The original source for this material is the AJCC Cancer Staging Manual, Sixth Edition (2002) published by Springer-Verlag New York, www.springer-ny.com.
The treatment of AdCC depends on the size and location of the tumor, whether the cancer has spread, and the person’s overall health. In many cases, a team of doctors will work with the patient to determine the best treatment plan.
This section outlines treatments that are the standard of care (the best treatments available) for this specific type of cancer. Patients are also encouraged to consider clinical trials when making treatment plan decisions. A clinical trial is a research study to test a new treatment to prove it is safe, effective, and possibly better than standard treatment. Your doctor can help you review all treatment options. For more information, visit the Clinical Trials section.
Surgery
Surgical removal of the tumor is the best treatment for AdCC, provided it can be done safely and is likely to have a good outcome. During surgery, the surgeon will remove the tumor and an area of tissue surrounding it, called a margin. If the margin removed during surgery is found to be “clean”, meaning at least 2 millimeters (mm) of tissue that is cancer-free surrounding the tumor, this signals total tumor removal and gives the best chance for successful treatment at the site of origin.
Side effects from surgery vary depending upon the surgical site. A concern with all surgeries of the parotid gland is that the surgery may damage or sever facial nerves, which can cause the muscles in the face to droop. Sometimes, a facial nerve graft may be used to restore function of the facial muscles after surgery to remove the tumor. The extent of surgery will vary by the site of the tumor and the structures that are involved.
External-beam radiation therapy
Radiation therapy is the use of high-energy x-rays or other particles to kill cancer cells. The most common type of radiation treatment is called external-beam radiation therapy, which is radiation given from a machine outside the body. Radiation therapy is particularly effective as an adjuvant therapy (therapy given in addition to the primary treatment) after surgery by helping to eliminate any microscopic cancer cells remaining in the body after surgery. Radiation therapy is not used pre-operatively (before surgery) for this disease, as many surgeons feel that the surgical wound heals better if radiation therapy is not given prior to surgery.
A specific method of external-beam radiation therapy, known as intensity modulated radiation therapy (IMRT), allows for more effective doses of radiation therapy to be delivered while reducing the damage to healthy cells and causing fewer side effects.
Since radiation therapy is targeted to a specific area, the side effects will depend on where the radiation therapy was given. Generally, during head and neck treatments, people may experience a sore mouth and/or throat that can cause pain and difficulty in swallowing. They may also have a skin reaction or burn that causes some pain. Because of the effects of radiation therapy on normal tissues, especially around the mouth and throat, patients are encouraged to receive prophylactic (preventive) care by a dentist trained in oncology before treatment begins.
Long-term side effects of radiation therapy vary with the sites involved, but they may include permanent loss of saliva causing dryness of the mouth and deterioration of the teeth. In some cases, a drug to stimulate the parotid saliva gland and increase or restore saliva levels may be given. Currently, amifostine (Ethyol), a drug to protect tissues during radiation therapy is being studied. Scarring or fibrosis (hardening or thickening) of the skin and muscles may develop several months to years after radiation therapy and lead to problems with wound healing, neck stiffness, and trouble swallowing. Other long-term effects that can develop include accelerated narrowing of the carotid arteries (the arteries that supply the head and neck with blood) and hypothyroidism (underactive thyroid disease).
Patients are encouraged to meet with a qualified speech pathologist prior to radiation therapy, so appropriate exercises can be performed to maintain the strength and flexibility of the muscles used during speech and swallowing.
Neutron and proton radiation therapy
Neutron therapy is a different form of radiation therapy. Compared with conventional radiation therapy, neutrons can deliver 20 times to 100 times more energy along their path length, causing much greater damage to the cancer cells. Neutron radiation therapy has been tested on many different types of tumors, with salivary gland tumors and, in particular, AdCC showing the greatest benefit. In certain instances, it may be the treatment of choice for AdCC, particularly in areas of the body where it is difficult to perform surgery. However, neutron therapy carries more severe side effects than conventional radiation therapy, such as severe sore mouth and/or throat and difficulty swallowing, and is therefore generally used more often with people with inoperable or recurrent disease. Medication to help increase saliva production and protect tissues may be given during this treatment. Neutrons cannot be used to treat large areas of the body for metastatic disease, but they can be used to treat isolated metastases that are causing problems, such as pain, blocking part of the lung, or pressing on the spinal cord.
A new type of radiation is proton therapy. Proton therapy acts similarly to traditional radiation therapy to kill cancer cells. The advantage of proton therapy is the ability to better control where the radiation is delivered, greatly reducing the damage to normal healthy tissues that surround the tumor. However, proton therapy has not been extensively investigated for use in AdCC.
Neither neutron nor proton therapy is widely available. Talk with your doctor for information about availability of these treatments.
Chemotherapy
Chemotherapy is the use of drugs to kill cancer cells. Chemotherapy is generally not used for AdCC, as this type of cancer appears to be less responsive to chemotherapy than other types of cancer. Various combinations of chemotherapy have been used to treat AdCC, without much success. The combination of chemotherapy and radiation therapy is considered experimental for AdCC.
Because AdCC is a rare disease, and, in general, the current chemotherapy is of limited benefit, it is recommended that people with AdCC ask about clinical trials to treat this disease. A second opinion at a cancer center may also be beneficial.
The medications used to treat cancer are continually being evaluated. Talking with your doctor is often the best way to learn about the medications you've been prescribed, their purpose, and their potential side effects or interactions with other medications. Learn more about your prescriptions through Cancer.Net's Drug Information Resources, which provides links to searchable drug databases.
Doctors and scientists are always looking for better ways to treat patients with AdCC. A clinical trial is a way to test a new treatment to prove that it is safe, effective, and possibly better than a standard treatment. Patients who participate in clinical trials are among the first to receive new treatments before they are widely available. However, there is no guarantee that the new treatment will be safe, effective, or better than a standard treatment.
Patients decide to participate in clinical trials for many reasons. For some patients, a clinical trial is the best treatment option available. Because standard treatments are not perfect, patients are often willing to face the added uncertainty of a clinical trial in the hope of a better result. Other patients volunteer for clinical trials because they know that finding new drugs and other therapies is the only way to make progress in treating AdCC. Even if they do not benefit directly from the clinical trial, their participation may benefit future patients with AdCC.
To join a clinical trial, patients must complete a learning process known as informed consent. During informed consent, the doctor should list all of the patient’s options, so the person understands how the new treatment differs from the standard treatment. The doctor must also list all of the risks of the new treatment, which may or may not be different than the risks of standard treatment. Finally, the doctor must explain what will be required of each patient in order to participate in the clinical trial, including the number of doctor visits, tests, and the schedule of treatment. Learn more about clinical trials, including patient safety, phases of a clinical trial, deciding to participate in a clinical trial, questions to ask the research team, and links to find cancer clinical trials.
Cancer and its treatment can cause a variety of side effects. However, doctors have made major strides in recent years in reducing pain, nausea and vomiting, and other physical side effects of cancer treatments. Many treatments used today are less intensive but as effective as treatments used in the past. Doctors also have many ways to provide relief to patients when such side effects do occur.
Fear of treatment side effects is common after a diagnosis of cancer, but it may be helpful to know that preventing and controlling side effects is a major focus of your health-care team. Before treatment begins, talk with your doctor about possible side effects of the specific treatments you will be receiving. The specific side effects that can occur depend on a variety of factors, including the type of cancer, its location, the individual treatment plan (including the length and dosage of treatment), and the person’s overall health.
Ask your doctor which side effects are most likely to happen (and which are not), when side effects are likely to occur, and how they will be addressed by the health-care team if they do happen. Also, be sure to communicate with the doctor about side effects you experience during and after treatment. For more information on the most common side effects of cancer and different treatments, along with ways to prevent or control them, visit Cancer.Net’s section on Managing Side Effects, based on ASCO’s curriculum.
In addition to physical side effects, there may be psychosocial (emotional and social) effects as well. Learn more about the importance of addressing these needs in Cancer.Net’s section on Caring for the Whole Patient.
For more information on late effects or long-term side effects, please read the After Treatment section or talk with your doctor.
After treatment for AdCC ends, talk with your doctor about developing a follow-up care plan. This plan may include regular physical examinations and/or medical tests to monitor your recovery for the coming months and years.
People treated with surgery may need rehabilitation to regain functioning and flexibility. Follow-up care by an oncologic dentist may be needed after radiation therapy. Sometimes, care may be given by a prosthodontist (a dental specialist with additional training and certification in the restoration and replacement of broken teeth with crowns, bridges, and removable prosthetics [dentures]) if major surgery to the mouth or jaw has taken place during treatment.
Intense treatments to the head and neck can affect swallowing, and patients may experience pain. These symptoms can be treated and, in most cases, subside some time after therapy.
Some people may experience depression following treatment, and this may require specialized therapy as well. Most cancer centers have support groups that encourage people to become more informed about these issues.
Periodically, people need follow-up care with their doctor to monitor the effects of treatment and to check for recurrences or distant metastases, which may occur even years after successful primary treatment. There is no known way to prevent recurrence of AdCC. A secondary cancer is rare in AdCC.
People recovering from AdCC are encouraged to follow established guidelines for good health, such as maintaining a healthy weight, not smoking, eating a balanced diet, and having recommended cancer screening tests. Talk with your doctor to develop a plan that is best for your needs. Moderate physical activity can help rebuild your strength and energy level. Your doctor can help you create an appropriate exercise plan based upon your needs, physical abilities, and fitness level. Learn more about Healthy Living After Cancer.
Research for AdCC is ongoing. The following advances may still be under investigation in clinical trials and may not be approved or available at this current time. Always discuss all diagnostic and treatment options with your doctor.
Antiangiogenesis research. Antiangiogenesis research, which explores how a tumor develops its blood supply and how agents disrupt these blood supplies that feed the tumor, may benefit people with AdCC.
Targeted therapies. New drugs called targeted therapies are designed to stop the growth of cancer cells at the level of genes and proteins. Currently, targeted therapies for cancer are being developed, and some of them may be useful in the treatment of AdCC.
Newer chemotherapy. Several clinical trials are examining the effects of relatively new chemotherapy alone, or in combination with other drugs, in the control of metastatic or locally recurrent AdCC.
Regular communication with your doctor is important in making informed decisions about your health care. Consider asking the following questions of your doctor:
Where exactly is the tumor located?
Can you describe the stage of the cancer? Is it possible to predict its future behavior?
Can you explain my pathology report to me?
What are my treatment options?
What clinical trials are open to me?
Which treatment option do you recommend? Why?
What are the possible side effects of this treatment, both in the short term and the long term?
How will this treatment affect my daily life? Will I be able to work, exercise, and perform my usual activities?
Will there be cosmetic changes to my appearance? How? For how long?
Will there be permanent issues relating to saliva production or eating?
Should I talk with an oncologic dentist before treatment begins?
Should I meet with a speech therapist before treatment begins?
How can I keep myself as healthy as possible during treatment?
What follow-up tests will I need, and how often will I need them?
What support services are available to me? To my family?
What are the chances this cancer will recur?
What treatments are available if my cancer recurs?
Printer-Friendly
E-mail this page