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Adenoid Cystic Carcinoma - Overview

This section has been reviewed and approved by the Cancer.Net Editorial Board, 09/2013

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. To see other pages, use the menu on the side of your screen. Think of that menu as a roadmap to this full guide.

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 that begins in glandular tissues. AdCC is found mainly in the head and neck, but it can occasionally occur in a woman’s uterus or other locations in the body. AdCC most commonly occurs in the salivary glands, which consist of clusters of cells that secrete saliva 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). 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)
    • Larynx (voicebox)
    • Trachea (windpipe)
  • Major salivary glands
    • Parotid (largest salivary gland found on either side of the face in front of each ear)
    • 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 variations of the tumor (what tumor cells look like under a microscope), and can be classified as cylindroma, cribiform, or solid AdCC. AdCC is sometimes classified as a disease of the minor salivary gland, even though it may begin at other sites.

This section covers AdCC. Learn more about other head and neck cancers.

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Adenoid Cystic Carcinoma - Statistics

This section has been reviewed and approved by the Cancer.Net Editorial Board, 09/2013

ON THIS PAGE:You will find information about how many people learn they have this type of cancer each year and some general survival information. Remember, survival rates depend on several factors. To see other pages, use the menu on the side of your screen.

AdCC is rare. AdCC is most often diagnosed in people in their 40s to 60s, but there are known cases of pediatric (childhood) AdCC. Each year, approximately 1,200 people are diagnosed with AdCC in the United States, and about 60% are women.

The five-year survival rate is the percentage of people who survive at least five years after the cancer is detected, excluding those who die from other diseases. The five year survival rate of people with AdCC is approximately 89%. The 15-year survival rate of people with AdCC is approximately 40%. Tumor growth for AdCC is often slow, and people 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 people with this type of cancer in the United States, 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 survival statistics are often measured in multi-year intervals, they may not represent advances made in the treatment or diagnosis of this cancer. Learn more about understanding statistics.

Sources:  Oral Cancer Foundation and Adenoid Cystic Carcinoma Research Foundation.

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Adenoid Cystic Carcinoma - Risk Factors

This section has been reviewed and approved by the Cancer.Net Editorial Board, 09/2013

ON THIS PAGE: You will find out more about what factors increase the chance of this type of cancer. To see other pages, use the menu on the side of your screen.

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 not known 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.       

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Adenoid Cystic Carcinoma - Symptoms and Signs

This section has been reviewed and approved by the Cancer.Net Editorial Board, 09/2013

ON THIS PAGE: You will find out more about body changes and other things that can signal a problem that may need medical care. To see other pages, use the menu on the side of your screen.

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
  • Difficulty swallowing
  • Hoarseness
  • Dull pain
  • 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.

The next section helps explain what tests and scans may be needed to learn more about the cause of the symptoms. Use the menu on the side of your screen to select Diagnosis, or you can select another section, to continue reading this guide.

Adenoid Cystic Carcinoma - Diagnosis

This section has been reviewed and approved by the Cancer.Net Editorial Board, 09/2013

ON THIS PAGE: You will find a list of the common tests, procedures, and scans that doctors can use to find out what’s wrong and identify the cause of the problem. To see other pages, use the menu on the side of your screen.

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 spread. This list describes options for diagnosing this type of cancer, and not all tests listed will be used for every person. Your doctor may consider these factors when choosing a diagnostic test:

  • Age and medical condition
  • Type of cancer suspected
  • Signs and 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 during 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 using a fine needle biopsy or by surgically removing 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 from the suspicious area. 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 (size and location) of the tumor before 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 or given orally (by mouth) 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. A CT scan can also be used to measure the tumor’s size. Sometimes, a contrast medium is injected into a patient’s vein or given orally 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.

After these diagnostic tests are done, your doctor will review all of the results with you. If the diagnosis is cancer, these results also help the doctor describe the cancer; this is called staging.

The next section helps explain the different stages for this type of cancer. Use the menu on the side of your screen to select Stages, or you can select another section, to continue reading this guide. 

Adenoid Cystic Carcinoma - Stages

This section has been reviewed and approved by the Cancer.Net Editorial Board, 09/2013

ON THIS PAGE: You will learn about how doctors describe a tumor’s growth or spread. This is called the stage. To see other pages, use the menu on the side of your screen.

Staging is a way of describing where a cancerous tumor is located, if or where it has spread, and whether it is affecting other parts of 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 standard staging system used for AdCC, but the staging system for a major salivary gland tumor is often used, which is based on the TNM system. 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)

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.

Here are more details on each part of the TNM system:

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: The primary tumor cannot be evaluated.

T0: No evidence of a tumor is found.

T1: The tumor is small, 2 centimeters (cm) at its widest dimension, and noninvasive (has not grown outside the area where it began).

T2: The tumor is larger, between 2 cm and 4 cm, but noninvasive.

T3: The tumor is larger than 4 cm, but not larger than 6 cm, and 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 has invaded the skin, jawbone, ear canal, and/or facial nerve.

T4b: The tumor has invaded 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: The neck has undergone an intervention that prevents the evaluation of lymph nodes.

N0: There is no evidence of cancer in the regional nodes.

N1: The 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: The 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: The 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: The cancer has spread to more than one lymph node on either side of the body, and no tumor measures larger than 6 cm.

N3: The cancer found in the 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: Distant metastasis cannot be evaluated.

M0: The cancer has not spread to other parts of the body.

M1: The cancer 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: This stage describes a noninvasive tumor (T1, T2) with no spread to lymph nodes (N0) and no distant metastasis (M0).

Stage II: This stage describes an invasive tumor (T3) with no spread to lymph nodes (N0) or distant metastasis (M0).

Stage III: This stage describes a smaller tumor (T1, T2) that has spread to regional lymph nodes (N1) but shows no sign of metastasis (M0).

Stage IVA: This stage describes any invasive tumor (T4a) that either has no lymph node involvement (N0) or has spread to only a single, same-sided lymph node (N1), but with no metastasis (M0). It is also used to describe a T3 tumor with one-sided nodal involvement (N1) but no metastasis (M0), or any tumor (any T) with extensive nodal involvement (N2) but no metastasis (M0).

Stage IVB: This stage describes any cancer (any T) with more extensive spread to lymph nodes (N2, N3) and no metastasis (M0).

Stage IVC: This stage describes any cancer (any T, any N) with distant metastasis (M1).

Recurrent: Recurrent cancer is cancer has come back after treatment. If there is a recurrence, the cancer may need to be staged again (called re-staging) using the system above.

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, Seventh Edition (2010) published by Springer-Verlag New York, www.cancerstaging.net.

Information about the cancer’s stage will help the doctor recommend a treatment plan. The next section helps explain the treatment options for this type of cancer. Use the menu on the side of your screen to select Treatment Options, or you can select another section, to continue reading this guide. 

Adenoid Cystic Carcinoma - Treatment Options

This section has been reviewed and approved by the Cancer.Net Editorial Board, 09/2013

ON THIS PAGE: You will learn about the different ways doctors use to treat people with this type of cancer. To see other pages, use the menu on the side of your screen.

This section outlines treatments that are the standard of care (the best proven treatments available) for this specific type of cancer. When making treatment plan decisions, patients are also encouraged to consider clinical trials as an option. A clinical trial is a research study to test a new treatment to evaluate whether it is safe, effective, and possibly better than the standard treatment. Your doctor can help you review all treatment options. For more information, visit the Clinical Trials and Latest Research sections.

Treatment overview

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. The team may include medical and radiation oncologists; surgeons; otolaryngologists (ear, nose, and throat doctors); plastic (reconstruction) surgeons; maxillofacial prosthodontists (specialists who perform restorative surgery in the head and neck areas); dentists; physical therapists; speech pathologists; audiologists (hearing experts); psychiatrists; nurses; dietitians; and social workers. It is extremely important that this 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.

Descriptions of the most common treatment options for AdCC are listed below. 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 your treatment options and be sure to ask questions about things that are unclear. Also, talk about the goals of each treatment with your doctor and what you can expect while receiving the treatment. Learn more about making treatment decisions.

Surgery

Surgery is the removal of the tumor and surrounding 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. A surgical oncologist is a doctor who specializes in treating cancer using surgery. 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 structures that are involved.

Because AdCC may frequently spread along nerves, the surgeon should examine and determine whether nerves are involved. Major nerves that are uninvolved 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.

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 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 used to restore the function of the facial muscles after surgery to remove the tumor. Patients should meet with different members of their health care team to help them make important decisions about treatment, to learn about programs to help them adjust to changes in their body and body image following treatment, and to understand what side effects to expect and how to cope with them. Learn more about cancer surgery.

Occasionally, it is not possible to remove AdCC using surgery. This type of tumor is called inoperable. In these cases, your doctor will recommend another treatment option (see below).

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. A doctor who specializes in giving radiation therapy to treat cancer is called a radiation oncologist. A radiation therapy regimen (schedule) usually consists of a specific number of treatments given over a set period of time.

Radiation therapy is particularly effective for AdCC as an adjuvant therapy (therapy given after surgery) by helping to eliminate any microscopic cancer cells that may remain 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.

Before beginning radiation treatment for any head and neck cancer, patients should receive a thorough examination from an oncologic dentist (a dentist experienced in treating people with head and neck cancer). 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.

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).

Learn more about 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 salivary gland tumors and AdCC have shown the greatest benefit. In certain instances, neutron radiation therapy 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; therefore, it is generally used more often with people with an inoperable tumor (surgery cannot be performed) 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, 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 proton therapy.

Neutron and proton therapy are not widely available. Talk with your doctor for information about the availability of these treatments.

Chemotherapy

Chemotherapy is the use of drugs to kill cancer cells, usually by stopping the cancer cells’ ability to grow and divide. Systemic chemotherapy is delivered through the bloodstream to reach cancer cells throughout the body. Chemotherapy is given by a medical oncologist, a doctor who specializes in treating cancer with medication. A chemotherapy regimen (schedule) usually consists of a specific number of cycles given over a set period of time.

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 tried 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. It may also be beneficial to get a second opinion regarding treatment options. Learn more about seeking a second opinion.

Getting care for symptoms and side effects

AdCC and its treatment often cause side effects. In addition to treatment to slow, stop, or eliminate the cancer, an important part of cancer care is relieving a person’s symptoms and side effects. This approach is called palliative or supportive care, and it includes supporting the patient with his or her physical, emotional, and social needs.

Palliative care can help a person at any stage of illness. People often receive treatment for the cancer and treatment to ease side effects at the same time. In fact, patients who receive both often have less severe symptoms, better quality of life, and report they are more satisfied with treatment.

Palliative treatments vary widely and often include medication, nutritional changes, relaxation techniques, and other therapies. You may also receive palliative treatments similar to those meant to eliminate the cancer, such as chemotherapy, surgery, and radiation therapy. Talk with your doctor about the goals of each treatment in the treatment plan.

Before treatment begins, talk with your health care team about the possible side effects of your specific treatment plan and supportive care options. And during and after treatment, be sure to tell your doctor or another health care team member if you are experiencing a problem so it is addressed as quickly as possible. Learn more about palliative care.

Recurrent AdCC

A remission is when cancer cannot be detected in the body and there are no symptoms. This may also be called “no evidence of disease” or NED. 

A remission can be temporary or permanent. This uncertainty leads to many survivors feeling worried or anxious that the cancer will come back. While many remissions are permanent, it’s important to talk with your doctor about the possibility of the cancer returning. Understanding the 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 does return after the original treatment, it is called recurrent cancer. It may come back in the same place (called a local recurrence), nearby (regional recurrence), or in another place (distant recurrence).

When this occurs, a cycle of testing will begin again to learn as much as possible about the recurrence, including whether the cancer’s stage has changed. After testing is done, you and your doctor will talk about your treatment options. Often the treatment plan will include the therapies 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 also suggest clinical trials that are studying new ways to treat this type of recurrent cancer.

People with recurrent cancer often experience emotions such as disbelief or fear. Patients are encouraged to talk with their health care team about these feelings and ask about support services to help them cope. Learn more about dealing with cancer recurrence.

Metastatic AdCC

If cancer has spread to another location in the body, it is called metastatic cancer. Patients with this diagnosis are encouraged to talk with doctors who are experienced in treating this stage of cancer, because there can be different opinions about the best treatment plan. Learn more about seeking a second opinion before starting treatment, so you are comfortable with the treatment plan chosen. This discussion may include clinical trials.

Your health care team may recommend a treatment plan that includes a combination of surgery and radiation therapy. Supportive care will also be important to help relieve symptoms and side effects.

For most patients, a diagnosis of metastatic cancer is very stressful and, at times, difficult to bear. Patients and their families are encouraged to talk about the way they are feeling with doctors, nurses, social workers, or other members of the health care team. It may also be helpful to talk with other patients, including through a support group.

If treatment fails

Recovery from cancer is not always possible. If treatment is not successful, the disease may be called advanced or terminal cancer.

This diagnosis is stressful, and this is difficult to discuss for many people. However, it is important to have open and honest conversations with your doctor and health care team to express your feelings, preferences, and concerns. The health care team is there to help, and many team members have special skills, experience, and knowledge to support patients and their families. Making sure a person is physically comfortable and free from pain is extremely important.

Patients who have advanced cancer and who are expected to live less than six months may want to consider a type of palliative care called 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 think about where you would be most comfortable: at home, in the hospital, or in a hospice environment. Nursing care and special equipment can make staying at home a workable alternative 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 bereavement.

The next section helps explain clinical trials, which are research studies. Use the menu on the side of your screen to select About Clinical Trials, or you can select another section, to continue reading this guide.

Adenoid Cystic Carcinoma - About Clinical Trials

This section has been reviewed and approved by the Cancer.Net Editorial Board, 09/2013

ON THIS PAGE: You will learn more about clinical trials, which are the main way that new medical approaches are tested to see how well they work. To see other pages, use the menu on the side of your screen.

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. To make scientific advances, doctors create research studies involving volunteers, called clinical trials.

Many clinical trials are focused on new treatments, evaluating whether a new treatment is safe, effective, and possibly better than the current (standard) treatment. These types of studies evaluate new drugs, different combinations of existing treatments, new approaches to radiation therapy or surgery, and new methods of treatment. Patients who participate in clinical trials are often 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.

There are also clinical trials that study new ways to ease symptoms and side effects during treatment and manage the late effects that may occur after treatment. Talk with your doctor about clinical trials regarding side effects. In addition, there are ongoing studies about ways to prevent the disease.

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 these studies are 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.

Sometimes people have concerns that, by participating in a clinical trial, they may receive no treatment by being given a placebo or a “sugar pill.” The use of placebos in cancer clinical trials is rare. When a placebo is used in a study, it is done with the full knowledge of the participants. Find out more about placebos in cancer clinical trials.

To join a clinical trial, patients must participate in a process known as informed consent. During informed consent, the doctor should list all of the patient’s options so that 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.

For specific topics being studied for AdCC, learn more in the Latest Research section.

Patients who participate in a clinical trial may stop participating at any time for any personal or medical reason. This may include that the new treatment is not working or there are serious side effects. Clinical trials are also closely monitored by experts who watch for any problems with each study. It is important that patients participating in a clinical trial talk with their doctor and researchers about who will be providing their treatment and care during the clinical trial, after the clinical trial ends, and/or if the patient chooses to leave the clinical trial before it ends. 

Cancer.Net offers a lot of information about cancer clinical trials in other areas of the website, including a complete section on clinical trials and places to search for clinical trials for a specific type of cancer.

The next section helps explain the areas of research going on today about this type of cancer. Use the menu on the side of your screen to select Latest Research, or you can select another section, to continue reading this guide.  

Adenoid Cystic Carcinoma - Latest Research

This section has been reviewed and approved by the Cancer.Net Editorial Board, 09/2013

ON THIS PAGE: You will read about the scientific research being done now to learn more about this type of cancer and how to treat it. To see other pages, use the menu on the side of your screen.

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 time. Always discuss all diagnostic and treatment options with your doctor.

Anti-angiogenesis research. Anti-angiogenesis therapy is a type of targeted therapy (see below). It is focused on stopping angiogenesis, which is the process of making new blood vessels. Because a tumor needs the nutrients delivered by blood vessels to grow and spread, the goal of anti-angiogenesis therapies is to “starve” the tumor. Research is focused on how this type of therapy may benefit patients with AdCC.

Targeted therapies. Targeted therapy is a treatment that targets the cancer’s specific genes, proteins, or the tissue environment that contributes to cancer growth and survival. Currently, targeted therapies are being developed that may be useful in the treatment of AdCC. In particular, research continues to determine the molecular mechanism that regulates the spread of AdCC along the nerves. Learn more about targeted treatments.

Chemotherapy. Several clinical trials are examining the effects of newer drugs alone, or in combination with other drugs, in the control of metastatic or locally recurrent AdCC.

Supportive care. Clinical trials are underway to find better ways of reducing symptoms and side effects of current AdCC treatments in order to improve patients’ comfort and quality of life.

To find clinical trials specific to your diagnosis, talk with your doctor or search online clinical trial databases now

The next section addresses how to cope with the symptoms of the disease or the side effects of its treatment. Use the menu on the side of your screen to select Coping with Side Effects, or you can select another section, to continue reading this guide.  

Adenoid Cystic Carcinoma - Coping with Side Effects

This section has been reviewed and approved by the Cancer.Net Editorial Board, 09/2013

ON THIS PAGE: You will find out more about steps to take to help cope with physical, social, and emotional side effects. This page includes several links outside of this guide to other sections of this website. To see other pages, use the menu on the side of your screen.

Fear of treatment side effects is common after a diagnosis of cancer, but it may help to know that preventing and controlling side effects is a major focus of your health care team. This is called palliative or supportive care, and it is an important part of the overall treatment plan, regardless of the stage of disease.

Common side effects from each treatment option for AdCC are described in detail within the Treatment Options section. Learn more about the most common side effects of cancer and different treatments, along with ways to prevent or control them. Side effects depend on a variety of factors, including the cancer’s stage, the length and dosage of treatment(s), and your overall health.

Before treatment begins, talk with your doctor about possible side effects of each type of treatment you will be receiving. Ask which side effects are most likely to happen, when they are likely to occur, and what can be done to prevent or relieve them. And, ask about the level of caregiving you may need during treatment and recovery, as family members and friends often play an important role in the care of a person with AdCC. Learn more about caregiving.

In addition to physical side effects, there may be psychosocial (emotional and social) effects as well. Patients and their families are encouraged to share their feelings with a member of their health care team who can help with coping strategies. Learn more about the importance of addressing such needs, including concerns about managing the cost of your cancer care.  

During and after treatment, be sure to tell the health care team about the side effects you experience, even if you feel they are not serious. Sometimes, side effects can last beyond the treatment period. A side effect that occurs months or years after treatment is called a long-term side effect or late effect. Treatment of both types of effects is an important part of survivorship care. Learn more by reading the After Treatment section or talking with your doctor.

The next section helps explain medical tests and check-ups needed after finishing cancer treatment. Use the menu on the side of your screen to select After Treatment, or you can select another section, to continue reading this guide.  

Adenoid Cystic Carcinoma - After Treatment

This section has been reviewed and approved by the Cancer.Net Editorial Board, 09/2013

ON THIS PAGE: You will read about your medical care after cancer treatment is finished and why this follow-up care is important. To see other pages, use the menu on the side of your screen.

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 should have regular follow-up check-ups 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 after AdCC.

ASCO offers cancer treatment summary forms to help keep track of the cancer treatment you received and develop a survivorship care plan once treatment is completed.

People treated with surgery may need cancer rehabilitation to regain physical function and flexibility. Treatments to the head and neck can affect swallowing, and patients may experience pain. These symptoms can be treated with rehabilitative therapy and, in most cases, get better gradually over time.

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.

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. Learn more about the next steps to take in survivorship, including making positive lifestyle changes.

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.

The next section offers a list of questions you may want to ask. Use the menu on the side of your screen to select Questions to Ask the Doctor, or you can select another section, to continue reading this guide.  

Adenoid Cystic Carcinoma - Questions to Ask the Doctor

This section has been reviewed and approved by the Cancer.Net Editorial Board, 09/2013

ON THIS PAGE: You will find some questions to ask your doctor or other members of your health care team, to help you better understand your diagnosis, treatment plan, and overall care. To see other pages, use the menu on the side of your screen.

Talking often with the doctor is important to make informed decisions about your health care. These suggested questions are a starting point to help you learn more about your cancer care and treatment. You are also encouraged to ask additional questions that are important to you. You may want to print this list and bring it to your next appointment, or download Cancer.Net’s free mobile app for an e-list and other interactive tools to manage your care.

  • Where exactly is the tumor located?
  • Can you describe the stage of the cancer? What does this mean?
  • Is it possible to predict its future behavior?
  • Can you explain my pathology report (laboratory test results) to me?
  • Did a pathologist with experience diagnosing salivary disease review my biopsy?
  • What clinical trials are open to me?
  • What treatment plan do you recommend? Why?
  • What is the goal of each treatment? Is it to eliminate the cancer, help me feel better, or both?
  • Who will be part of my health care team, and what does each member do?
  • How much experience do they have treating this disease?
  • Who will be coordinating my overall treatment and follow-up care?
  • 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 changes to my appearance? How? For how long? Will I need reconstructive surgery or other procedures?
  • 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 pathologist before treatment begins?
  • How can I keep myself as healthy as possible during treatment?
  • Could this treatment affect my sex life? If so, how and for how long?
  • Could this treatment affect my ability to become pregnant or have children? If so, should I talk with a fertility specialist before cancer treatment begins?
  • If I’m worried about managing the costs related to my cancer care, who can help me with these concerns?
  • What follow-up tests will I need, and how often will I need them?
  • What are the chances this cancer will recur?
  • What treatments are available if my cancer recurs?
  • What support services are available to me? To my family?
  • Whom should I call for questions or problems?

The next section offers some more resources that may be helpful to you. Use the menu on the side of your screen to select Additional Resources, or you can select another section, to continue reading this guide.  

Adenoid Cystic Carcinoma - Additional Resources

This section has been reviewed and approved by the Cancer.Net Editorial Board, 09/2013

ON THIS PAGE: You will find some helpful links to other areas of Cancer.Net that provide information about cancer care and treatment. This is the final page of Cancer.Net’s Guide to Adenoid Cystic Carcinoma. To go back and review other pages, use the menu on the side of your screen.

Cancer.Net includes many other sections about the medical and emotional aspects of cancer, both for the person diagnosed and their family members and friends. This website is meant to be a resource for you and your loved ones from the time of diagnosis, through treatment, and beyond. Here are a few sections that may get you started in exploring the rest of Cancer.Net:

This is the end of Cancer.Net’s Guide to Adenoid Cystic Carcinoma. Use the menu on the side of your screen to select another section, to continue reading this guide.