Adenoid cystic carcinoma (ACC) is not considered to be an inherited cancer. Like most cancers, ACC appears to develop from genetic changes that are caused by something in a person's environment. In almost all reported cases, ACC has occurred sporadically in people with no family history of ACC. However, we are aware of two reports in the literature in which more than one family member had ACC. There has also been speculation about whether ACC may be associated with certain family cancer syndromes, but more research is needed to determine if an association exists.

Tests that examine the head, neck, and the inside of the mouth are used to diagnose salivary gland cancer.

The following procedures may be used:

• Physical exam and history: An exam of the body to check general signs of health. The head, neck, mouth, and throat will be checked for signs of disease, such as lumps or anything else that seems unusual. A history of the patient's health habits and past illnesses and treatments will also be taken.
• MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI).
• CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.

Image Caption : Computed tomography (CT) scan of the head and neck. The patient lies on a table that slides through the CT scanner, which takes x-ray pictures of the inside of the head and neck.

• PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do.
• Endoscopy: A procedure to look at organs and tissues inside the body to check for abnormal areas. For salivary gland cancer, an endoscope is inserted into the mouth to look at the mouth, throat, and larynx. An endoscope is a thin, tube-like instrument with a light and a lens for viewing.
• Biopsy: The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer.
  • Fine needle aspiration (FNA) biopsy: The removal of tissue or fluid using a thin needle. An FNA is the most common type of biopsy used for salivary gland cancer.
  • Incisional biopsy: The removal of part of a lump or a sample of tissue that doesn’t look normal.
  • Surgery: If cancer cannot be diagnosed from the sample of tissue removed during an FNA biopsy or an incisional biopsy, the mass may be removed and checked for signs of cancer.

Because salivary gland cancer can be hard to diagnose, patients should ask to have the tissue samples checked by a pathologist who has experience in diagnosing salivary gland cancer.

Certain factors affect treatment options and prognosis (chance of recovery).

The treatment options and prognosis depend on the following:

• The stage of the cancer (especially the size of the tumor).
• The type of salivary gland the cancer is in.
• The type of cancer cells (how they look under a microscope).
• The patient's age and general health.

There are different types of treatment for patients with salivary gland cancer.

Different types of treatment are available for patients with salivary gland cancer. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.

Patients with salivary gland cancer should have their treatment planned by a team of doctors who are experts in treating head and neck cancer.

Your treatment will be overseen by a medical oncologist, a doctor who specializes in treating people with cancer. Because the salivary glands help in eating and digesting food, patients may need special help adjusting to the side effects of the cancer and its treatment. The medical oncologist may refer you to other doctors who have experience and expertise in treating patients with head and neck cancer and who specialize in certain areas of medicine. These include the following:

• Head and neck surgeon.
• Radiation oncologist.
• Dentist.
• Speech therapist.
• Dietitian.
• Psychologist.
• Rehabilitation specialist.
• Plastic surgeon.

Three types of standard treatment are used:

Surgery

Surgery (removing the cancer in an operation) is a common treatment for salivary gland cancer. A doctor may remove the cancer and some of the healthy tissue around the cancer. In some cases, a lymphadenectomy (surgery in which lymph nodes are removed) will also be done.

After the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given radiation therapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy.

Radiation therapy

Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing.

• External radiation therapy uses a machine outside the body to send radiation toward the area of the body with cancer.

Image Caption: External-beam radiation therapy of the head and neck. A machine is used to aim high-energy radiation at the cancer. The machine can rotate around the patient, delivering radiation from many different angles to provide highly conformal treatment. A mesh mask helps keep the patient’s head and neck from moving during treatment. Small ink marks are put on the mask. The ink marks are used to line up the radiation machine in the same position before each treatment.

• Special types of external radiation may be used to treat some salivary gland tumors. These include:

  • Fast neutron radiation therapy: Fast neutron radiation therapy is a type of high-energy external radiation therapy. A radiation therapy machine aims neutrons (tiny, invisible particles) at the cancer cells to kill them. Fast neutron radiation therapy uses a higher-energy radiation than the x-ray type of radiation therapy. This allows the radiation therapy to be given in fewer treatments.
  • Photon-beam radiation therapy: Photon-beam radiation therapy is a type of external radiation therapy that reaches deep tumors with high-energy x-rays made by a machine called a linear accelerator. This can be delivered as hyperfractionated radiation therapy, in which the total dose of radiation is divided into small doses and the treatments are given more than once a day.

External radiation therapy is used to treat salivary gland cancer, and may also be used as palliative therapy to relieve symptoms and improve quality of life.

Chemotherapy

Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy).

New types of treatment are being tested in clinical trials.

This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website.

Radiosensitizers

Radiosensitizers are drugs that make tumor cells more sensitive to radiation therapy. Combining radiation therapy with radiosensitizers may kill more tumor cells.

Treatment for salivary gland cancer may cause side effects.

Patients may want to think about taking part in a clinical trial.

For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment.

Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment.

Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.

Patients can enter clinical trials before, during, or after starting their cancer treatment.

Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment.

Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website.

Follow-up tests may be needed.

Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests.

Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.

Treatment of Stage I Salivary Gland Cancer

Treatment for stage I salivary gland cancer depends on whether the cancer is low-grade (slow growing) or high-grade (fast growing).

If the cancer is low-grade, treatment may include the following:

• Surgery with or without radiation therapy.
• Fast neutron radiation therapy.

If the cancer is high-grade, treatment may include the following:

• Surgery with or without radiation therapy.
• A clinical trial of chemotherapy.
• A clinical trial of a new local therapy.

Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.

Treatment of Stage II Salivary Gland Cancer

Treatment for stage II salivary gland cancer depends on whether the cancer is low-grade (slow growing) or high-grade (fast growing).

If the cancer is low-grade, treatment may include the following:

• Surgery with or without radiation therapy.
• Radiation therapy.
• Chemotherapy.

If the cancer is high-grade, treatment may include the following:

• Surgery with or without radiation therapy.
• Fast neutron or photon-beam radiation therapy.
• A clinical trial of radiation therapy and/or radiosensitizers.
• A clinical trial of chemotherapy.

Treatment of Stage III Salivary Gland Cancer

Treatment for stage III salivary gland cancer depends on whether the cancer is low-grade (slow growing) or high-grade (fast growing).

If the cancer is low-grade, treatment may include the following:

• Surgery with or without lymphadenectomy. Radiation therapy may also be given after surgery.
• Radiation therapy.
• Fast neutron radiation therapy to lymph nodes with cancer.
• Chemotherapy.
• A clinical trial of fast neutron radiation therapy to the tumor.
• A clinical trial of chemotherapy.

If the cancer is high-grade, treatment may include the following:

• Surgery with or without lymphadenectomy. Radiation therapy may also be given after surgery.
• Fast neutron radiation therapy.
• Radiation therapy as palliative therapy to relieve symptoms and improve quality of life.
• A clinical trial of radiation therapy and/or radiosensitizers.
• A clinical trial of chemotherapy.

Treatment of Stages IVA, IVB, and IVC Salivary Gland Cancer

Treatment of stage IVA, stage IVB, and stage IVC salivary gland cancer may include the following:

• Fast neutron or photon-beam radiation therapy.
• A clinical trial of chemotherapy with or without radiation therapy.

Treatment of Recurrent Salivary Gland Cancer

Treatment of recurrent salivary gland cancer may include the following:

• Radiation therapy.
• A clinical trial of a new treatment.