ON THIS PAGE: You will learn about the different types of treatments doctors use for people with adenoid cystic carcinoma (AdCC). Use the menu to see other pages.
This section explains the types of treatments, also known as therapies, that are the standard of care for AdCC. “Standard of care” means the best treatments known. When making treatment plan decisions, you are encouraged to discuss with your doctor whether clinical trials are an option. A clinical trial is a research study that tests a new approach to treatment. Doctors want to learn through clinical trials whether a new treatment is safe, effective, and possibly better than the standard treatment. Clinical trials can test a new drug, a new combination of standard treatments, or new doses of standard drugs or other treatments. Clinical trials are an option for all stages of cancer. Your doctor can help you consider all your treatment options. Learn more about clinical trials in the About Clinical Trials and Latest Research sections of this guide.
How adenoid cystic carcinoma is treated
In cancer care, different types of doctors often work together to create a patient’s overall treatment plan that combines different types of treatments. This is called a multidisciplinary team. Cancer care teams include a variety of health care professionals, including physicians, physician assistants, nurse practitioners, oncology nurses, social workers, pharmacists, counselors, dietitians, and others.
People with AdCC may work with physicians and other health care professionals in different specialties. These include:
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Medical oncologist: a doctor who specializes in treating cancer with medication
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Radiation oncologist: a doctor who specializes in giving radiation therapy to treat cancer
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Surgical oncologist: a doctor who specializes in treating cancer using surgery
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Maxillofacial prosthodontist: a specialist who performs restorative surgery in the head and neck areas
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Otolaryngologist: a doctor who specializes in the ear, nose, and throat
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Plastic, or reconstructive, surgeon: a surgeon who specializes in the reconstruction of facial and body tissue and the improvement in a person's appearance
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Oncologic dentist or oral oncologist: dentists experienced in caring for people with head and neck cancer
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Audiologist: a health professional who specializes in hearing and balance problems
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Physical therapist: a health professional who addresses functional challenges that may develop as a result of cancer and cancer treatment
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Speech pathologist: a specialist who helps people use muscles in the mouth and throat
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Mental health providers: health professionals who diagnose and treat mental health problems, such as a psychiatrist or a psychologist
It is extremely important that the cancer care team creates a comprehensive treatment plan before treatment begins, and people may need to be seen by several specialists before a treatment plan is fully developed.
Although eliminating the cancer is the primary goal of treatment, preserving the function of the nearby nerves, organs, and tissues is also very important. When planning treatment, doctors consider how it might affect a person’s quality of life, such as how a person feels, looks, talks, eats, and breathes.
Treatment options and recommendations depend on several factors, including the type and stage of cancer, possible side effects, and the patient's preferences and overall health. Take time to learn about all of your treatment options and be sure to ask questions about things that are unclear. Talk with your doctor about the goals of each treatment and what you can expect while receiving the treatment. These types of talks are called "shared decision-making." Shared decision-making is when you and your doctors work together to choose treatments that fit the goals of your care. Learn more about making treatment decisions.
Common types of treatments used for AdCC are described below, including surgery, radiation therapy, and systemic therapies using medication. Your care plan may also include treatment for your symptoms and side effects, an important part of cancer care.
Surgery
Surgery is the removal of the tumor and some surrounding healthy tissue during an operation.
Surgical removal of the tumor is the best treatment for AdCC as long as 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 healthy tissue surrounding it, called a margin. Surgery is considered the most successful if the margin removed during surgery is found to be “clean,” which means there is a minimum of 2 millimeters (mm) of cancer-free tissue surrounding the tumor. The extent of surgery will vary depending on the location of the tumor and the area of the body involved.
Because AdCC may frequently spread along nerves, the surgeon should examine and determine whether nerves are involved. Major nerves that are not involved should be identified and spared during surgery. Smaller branches of nerves should be analyzed to determine if the cancer has spread to the space surrounding the nerve. For a later-stage tumor or one that significantly involves nerves, the surgeon should remove as much of the tumor as possible while sparing the organs and functions of the face. These are difficult decisions to make. Talk with your doctor about how to find a surgeon with the necessary level of expertise, experience, and training to perform this type of surgery.
Side effects from surgery vary depending upon the surgical site. For example, surgery of the parotid salivary gland may damage facial nerves, which can cause the muscles in the face to droop. Sometimes, a facial nerve graft or other reconstructive procedure (plastic surgery) may be recommended to restore the function of the facial muscles after surgery to remove the tumor. Learn more about the basics of surgical reconstruction.
Before surgery, talk with your health care team about the possible side effects from the specific surgery you will have and whether reconstruction will be needed. Learn more about the basics of cancer surgery.
Additional cancer treatment may be needed after surgery, called adjuvant therapy. Occasionally, it is not possible to remove AdCC using surgery. This type of tumor is called inoperable or unresectable. In these cases, your doctor will recommend other treatment options, such as radiation therapy or cancer medication (see below).
External-beam radiation therapy
Radiation therapy is the use of high-energy x-rays or other particles to destroy cancer cells.
The most common type of radiation therapy is called external-beam radiation therapy, which is radiation given from a machine outside the body. A radiation therapy regimen, or schedule, usually consists of a specific number of treatments given over a set period.
Radiation therapy is particularly effective for AdCC as an adjuvant therapy by helping to eliminate any microscopic cancer cells that may remain in the body after surgery. Adjuvant therapy is cancer treatment given after surgery. In general, radiation therapy is not used before surgery for this disease. That is because 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 over time.
Before beginning radiation therapy for any head and neck cancer, people should receive a thorough examination from an oncologic dentist. Since radiation therapy can cause tooth decay, damaged teeth may need to be removed. Often, tooth decay can be prevented with proper treatment from a dentist before beginning treatment. Learn more about dental and oral health during cancer treatment.
People should also receive an evaluation from a speech pathologist with experience treating people with head and neck cancer. The speech pathologist can teach exercises that will help maintain the strength and flexibility of the muscles used during speech and swallowing. If side effects are affecting a person's nutrition or weight, talk with a registered dietitian nutritionist (RDN) about coping with eating challenges.
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 salivary 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, which is the 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, which supply the head and neck with blood. There may also be lower thyroid gland functioning, called hypothyroidism, which can be managed with thyroid hormone replacement medication. Learn more about the basics of radiation therapy.
Neutron and proton radiation therapy
Neutron therapy is a different form of radiation therapy that uses neutrons rather than x-rays. 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, and its treatment for salivary gland tumors and AdCC has shown the greatest benefit.
In certain instances, neutron radiation therapy may be the first treatment for AdCC, particularly in areas of the body where it is difficult to perform surgery. However, neutron therapy carries more severe side effects than traditional radiation therapy, such as severe sore mouth and/or throat and difficulty swallowing; therefore, it is generally used more often with people with an inoperable tumor or if the tumor recurs. 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 if there is widespread metastatic disease. Neutrons can be used to treat a single metastasis that is causing problems, such as pain, blockage of part of the lung, or pressure on the spinal cord.
Proton therapy, also called proton beam therapy, is a type of external-beam radiation therapy that uses protons rather than x-rays. At high energy, protons can destroy 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 researched for use in AdCC. Learn more about the basics of proton therapy.
Neutron and proton therapy are not widely available. Talk with your doctor for information about the availability of these treatments if recommended for you.
Physical, emotional, and social effects of cancer
Cancer and its treatment cause physical symptoms and side effects, as well as emotional, social, and financial effects. Managing all of these effects is called palliative care or supportive care. It is an important part of your care that is included along with treatments intended to slow, stop, or eliminate the cancer.
Palliative care focuses on improving how you feel during treatment by managing symptoms and supporting patients and their families with other, non-medical needs. Any person, regardless of age or type and stage of cancer, may receive this type of care. And it often works best when it is started right after a cancer diagnosis. People who receive palliative care along with treatment for the cancer often have less severe symptoms, better quality of life, and report that they are more satisfied with treatment.
Palliative treatments vary widely and often include medication, nutritional changes, relaxation techniques, emotional and spiritual support, and other therapies. You may also receive palliative treatments similar to those meant to get rid of the cancer, such as chemotherapy, surgery, or radiation therapy.
Before treatment begins, talk with your doctor about the goals of each treatment in the treatment plan being recommended. You should also talk about the possible side effects of the specific treatment plan and palliative care options. Many patients also benefit from talking with a social worker and participating in support groups. Ask your doctor about these resources, too.
During treatment, your health care team may ask you to answer questions about your symptoms and side effects and to describe each problem. Be sure to tell the health care team if you are experiencing a problem. This helps the health care team treat any symptoms and side effects as quickly as possible. It can also help prevent more serious problems in the future.
Learn more about the importance of tracking side effects in another part of this guide. Learn more about palliative care in a separate section of this website.
Metastatic adenoid cystic carcinoma
If cancer spreads to another part in the body from where it started, doctors call it metastatic cancer. If this happens, it is a good idea to talk with doctors who have experience in treating it. Doctors can have different opinions about the best standard treatment plan. Close observation may be recommended, especially if the disease is slow growing and causing no symptoms. Clinical trials might also be an option. Learn more about getting a second opinion before starting treatment, so you are comfortable with your chosen treatment plan.
Your treatment plan may include a combination of surgery and radiation therapy. Palliative care will also be important to help relieve symptoms and side effects.
For most people, a diagnosis of metastatic cancer is very stressful and difficult to bear. You and your family are encouraged to talk about how you feel with doctors, nurses, social workers, or other members of your health care team. It may also be helpful to talk with other patients, such as through a support group or other peer support program.
Therapies using medication to treat metastatic AdCC
The treatment plan may include medications to destroy cancer cells. Medication may be given through the bloodstream to reach cancer cells throughout the body. When a drug is given this way, it is called systemic therapy. Medication may also be given locally, which is when the medication is applied directly to the cancer or kept pin a single part of the body.
This treatment is generally prescribed by a medical oncologist, a doctor who specializes in treating cancer with medication.
Medications are often given through an intravenous (IV) tube placed into a vein using a needle or as a pill or capsule that is swallowed (orally). If you are given oral medications, be sure to ask your health care team about how to safely store and handle them.
The types of medications used for AdCC include:
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Chemotherapy
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Targeted therapy
Each of these types of therapies is discussed below in more detail. A person may receive 1 type of medication at a time or a combination of medications given at the same time. They can also be given as part of a treatment plan that includes surgery and/or radiation therapy.
The medications used to treat cancer are continually being evaluated. Talking with your doctor is often the best way to learn about the medications prescribed for you, their purpose, and their potential side effects or interactions with other medications.
It is also important to let your doctor know if you are taking any other prescription or over-the-counter medications or supplements. Herbs, supplements, and other drugs can interact with cancer medications, causing unwanted side effects or reduced effectiveness. Learn more about your prescriptions by using searchable drug databases.
Chemotherapy for metastatic AdCC
Chemotherapy is the use of drugs to destroy cancer cells, usually by keeping the cancer cells from growing, dividing, and making more cells. A chemotherapy regimen, or schedule, usually consists of a specific number of cycles given over a set period. A patient may receive 1 drug at a time or combinations of different drugs given at the same time. Learn more about the basics of chemotherapy and preparing for treatment.
In general, chemotherapy is usually 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 tried without much success. Researchers are looking at the combination of chemotherapy and radiation therapy, but this is considered experimental for AdCC.
Targeted therapy for metastatic AdCC
Targeted therapy is a treatment that targets the cancer’s specific genes, proteins, or the tissue environment that contributes to cancer growth and survival. This type of treatment blocks the growth and spread of cancer cells and limits damage to healthy cells.
Not all tumors have the same targets. To find the most effective treatment, your doctor may run tests to identify the genes, proteins, and other factors in your tumor. This helps doctors better match each patient with the most effective treatment whenever possible. In addition, research studies continue to find out more about specific molecular targets and new treatments directed at them. Learn more about the basics of targeted treatments.
For AdCC, clinical trials are researching the use of targeted therapy drugs called tyrosine kinase inhibitors (TKIs), such as lenvatinib (Lenvima), or axitinib (Inlyta), taken as oral medication. This has shown to be a beneficial approach for a small proportion of people with AdCC. Because of the general rarity of AdCC and that current chemotherapy is of limited benefit, it is recommended that people with AdCC ask about clinical trials to treat this disease.
It may also be beneficial to get a second opinion regarding AdCC treatment options. Learn more about seeking a second opinion.
Remission and the chance of recurrence
A remission is when cancer cannot be detected in the body and there are no symptoms. This may also be called having “no evidence of disease” or NED.
A remission may be temporary or permanent. This uncertainty causes many people to worry that the cancer will come back. While many remissions are permanent, it is important to talk with your doctor about the possibility of the cancer returning. Understanding your risk of recurrence and the treatment options may help you feel more prepared if the cancer does return. Learn more about coping with the fear of recurrence.
If the cancer returns after the original treatment, it is called recurrent cancer. It may come back in the same place, meaning it is a local recurrence, or nearby, which is a regional recurrence. If it comes back in another place, it is a distant recurrence.
If a recurrence happens, a new cycle of testing will begin again to learn as much as possible about it. After this testing is done, you and your doctor will talk about the treatment options. Often the treatment plan will include the treatments described above, such as surgery and radiation therapy, but they may be used in a different combination or given at a different pace. Your doctor may suggest clinical trials that are studying new ways to treat recurrent AdCC. Whichever treatment plan you choose, palliative care will be important for relieving symptoms and side effects.
People with recurrent cancer sometimes experience emotions such as disbelief or fear. You are encouraged to talk with your health care team about these feelings and ask about support services to help you cope. Learn more about dealing with cancer recurrence.
If treatment does not work
Recovery from cancer is not always possible. If the cancer cannot be cured or controlled, the disease may be called advanced or terminal.
This diagnosis is stressful, and for some people, advanced cancer is difficult to discuss. However, it is important to have open and honest conversations with your health care team to express your feelings, preferences, and concerns. The health care team has special skills, experience, and knowledge to support patients and their families and is there to help. Making sure a person is physically comfortable, free from pain, and emotionally supported is extremely important.
People who have advanced cancer and who are expected to live less than 6 months may want to consider hospice care. Hospice care is designed to provide the best possible quality of life for people who are near the end of life. You and your family are encouraged to talk with the health care team about hospice care options, which include hospice care at home, a special hospice center, or other health care locations. Nursing care and special equipment can make staying at home a workable option for many families. Learn more about advanced cancer care planning.
After the death of a loved one, many people need support to help them cope with the loss. Learn more about grief and loss.
The next section in this guide is About Clinical Trials. It offers more information about research studies that are focused on finding better ways to care for people with cancer. Use the menu to choose a different section to read in this guide.