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Adenoid Cystic Carcinoma - Introduction

This section has been reviewed and approved by the Cancer.Net Editorial Board, 01/2016

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. Think of that menu as a roadmap to this full guide.

Cancer begins when healthy cells change and grow out of control, forming a mass called a tumor. A tumor can be cancerous or benign. A cancerous tumor is malignant, meaning it can grow and spread to other parts of the body. A benign tumor means the tumor can grow but will not spread.

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 other locations in the body, including a woman’s uterus. AdCC most commonly occurs in the salivary glands, which consist of clusters of cells that secrete saliva scattered throughout the upper aerodigestive tract. The upper aerodigestive tract includes the organs and tissues of the upper respiratory tract, such as 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 — an 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 — the inner lining of the mouth; glands located here produce mucus

    • Larynx — the voice box

    • Trachea — the windpipe

  • Major salivary glands

    • Parotid — the largest salivary gland found on either side of the face in front of each ear

    • Submandibular — found under the jawbone

    • Sublingual glands — located in the bottom of the mouth under the tongue

Regardless of where it starts, AdCC tends to spread along nerves, known as a 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, which is the spread of cancer to another part of the body, is the lung. AdCC is known for having long periods of no growth, or indolence, 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, meaning what tumor cells look like under a microscope. The tumor 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.

The next section in this guide is Statistics. It helps explain how many people are diagnosed with this disease and general survival rates. Or, use the menu to choose another section to continue reading this guide.  

Adenoid Cystic Carcinoma - Statistics

This section has been reviewed and approved by the Cancer.Net Editorial Board, 01/2016

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

AdCC is rare. Each year, about 1,200 people are diagnosed with AdCC in the United States, and about 60% are women. AdCC is most often found in younger and middle-aged adults, but anyone of any age can be diagnosed, including children.

The 5-year survival rate tells you what percent of people live at least 5 years after the cancer is found. Percent means how many out of 100. The 5-year survival rate for 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 of AdCC is common and can occur many years after initial treatment. A late recurrence is cancer that has come back after treatment.

It is important to remember that statistics on how many people survive this type of cancer are an estimate. The estimate comes from data based on people with this cancer in the United States each year. So, your own risk may be different. Doctors cannot say for sure how long anyone will live with AdCC. Also, experts measure the survival statistics every 5 years. This means that the estimate may not show the results of better diagnosis or treatment available for less than 5 years. Learn more about understanding statistics.

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

The next section in this guide is Risk Factors. It explains what factors may increase the chance of developing this disease. Or, use the menu to choose another section to continue reading this guide.

Adenoid Cystic Carcinoma - Risk Factors

This section has been reviewed and approved by the Cancer.Net Editorial Board, 01/2016

ON THIS PAGE: You will find out more about the factors that increase the chance of developing this type of cancer. To see other pages, use the menu.

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 is somehow inactivated in advanced and aggressive forms of AdCC. The p53 gene limits cell growth by monitoring the rate at which cells divide.

The next section in this guide is Symptoms and Signs. It explains what body changes or medical problems this disease can cause. Or, use the menu to choose another section to continue reading this guide.

Adenoid Cystic Carcinoma - Symptoms and Signs

This section has been reviewed and approved by the Cancer.Net Editorial Board, 01/2016

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.

People with AdCC may experience the following symptoms or signs. Sometimes, people with AdCC do not have any of these changes. Or, the cause of a symptom may be another medical condition that is not cancer.

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

If you are concerned about any changes you experience, please talk with your doctor. Your doctor will ask how long and how often you have been experiencing the symptom(s), in addition to other questions. This is to help find out the cause of the problem, called a diagnosis.

If cancer is diagnosed, relieving symptoms 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 in this guide is Diagnosis. It explains what tests may be needed to learn more about the cause of the symptoms. Or, use the menu to choose another section to continue reading this guide.  

Adenoid Cystic Carcinoma - Diagnosis

This section has been reviewed and approved by the Cancer.Net Editorial Board, 01/2016

ON THIS PAGE: You will find a list of common tests, procedures, and scans that doctors use to find the cause of the problem. To see other pages, use the menu.

Doctors use many tests to find, or diagnose, cancer. They also do tests to learn if cancer has spread to another part of the body from where it started. If this happens, it is called metastasis. For example, imaging tests can show if the cancer has spread. Imaging tests show pictures of the inside of the body. Doctors may also do tests to learn which treatments could work best.

For most types of cancer, a biopsy is the only sure way for the doctor to know whether an area of the body has cancer. In a biopsy, the doctor takes a small sample of tissue for testing in a laboratory. If a biopsy is not possible, the doctor may suggest other tests that will help make a diagnosis.

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:

  • The type of cancer suspected

  • Your signs and symptoms

  • Your age and medical condition

  • The results of earlier medical tests

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. A pathologist then analyzes the sample(s).  A pathologist is 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 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. MRI can also be used to measure the tumor’s size. A special dye called a contrast medium is given before the scan to create a clearer picture. This dye can be injected into a patient’s vein or given as a pill to swallow. An MRI is very useful for identifying perineural spread of AdCC. Perineural spread is growth of the tumor along nerve branches.

    • A CT scan creates a 3-dimensional picture of the inside of the body using x-rays taken from different angles. 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 special dye called a contrast medium is given before the scan to provide better detail on the image. This dye can be injected into a patient’s vein or given as a pill to swallow.

    • A PET scan is a way to create pictures of organs and tissues inside the body. A PET scan is usually combined with a CT scan (see above), called a PET-CT scan. However, you may hear your doctor refer to this procedure just as a PET scan. A small amount of a radioactive sugar substance is injected into the patient’s body. This sugar substance is taken up by cells 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 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 in this guide is Stages. It explains the system doctors use to describe the extent of the disease. Or, use the menu to choose another section to continue reading this guide.  

Adenoid Cystic Carcinoma - Stages

This section has been reviewed and approved by the Cancer.Net Editorial Board, 01/2016

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.

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 find out 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, which is the chance of recovery. There are different stage descriptions for different types of cancer.

TNM staging system

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. Doctors use the results from diagnostic tests and scans to answer these questions:

  • Tumor (T): How large is the primary tumor? Where is it located?

  • Node (N): Has the tumor spread to the lymph nodes? If so, where and how many? 

  • Metastasis (M): Has the cancer metastasized to other parts of the body? If so, where and how much?

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 (T)

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. It is also noninvasive, which means it 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.

Tumor (T)

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.

Metastasis (M)

The "M" in the TNM system describes cancer that has spread to other parts of the body, called distant metastatsis.

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 or 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 or 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 or 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 the cancer does return, there will be another round of tests to learn about the extent of the recurrence. These tests and scans are often similar to those done at the time of the original diagnosis.

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 published by Springer-Verlag New York, www.cancerstaging.net.

Information about the cancer’s stage will help the doctor recommend a specific treatment plan. The next section in this guide is Treatment Options. Or, use the menu to choose 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, 01/2016

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.

This section tells you the treatments that are the standard of care for this type of cancer. “Standard of care” means the best treatments known. When making treatment plan decisions, patients are also encouraged to consider clinical trials as an option. A clinical trial is a research study that tests a new approach to treatment. Doctors want to learn if it 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. Your doctor can help you consider all your treatment options. To learn more about clinical trials, see the About 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, who are ear, nose, and throat doctors; plastic, or reconstruction, surgeons; maxillofacial prosthodontists, specialists who perform restorative surgery in the head and neck areas; dentists; physical therapists; speech pathologists; audiologists, or 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. You care plan may also include treatment for your symptoms and side effects, an important part of cancer care. Take time to learn about all of 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 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. 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 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 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 the basics of 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 for more information.

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 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, which is your treatment 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 by helping to eliminate any microscopic cancer cells that may remain in the body after surgery. Adjuvant therapy is therapy given after surgery. Radiation therapy is not used pre-operatively, meaning 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.

Before beginning radiation treatment for any head and neck cancer, patients should receive a thorough examination from an oncologic dentist. An oncologic dentist is 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, 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, and underactive thyroid disease called hypothyroidism. 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 salivary gland tumors and AdCC has 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 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.

Chemotherapy

Chemotherapy is the use of drugs to destroy cancer cells, usually by stopping the cancer cells’ ability to grow and divide. Chemotherapy is given by a medical oncologist, a doctor who specializes in treating cancer with medication.

Systemic chemotherapy gets into the bloodstream to reach cancer cells throughout the body. Common ways to give chemotherapy include an intravenous (IV) tube placed into a vein using a needle or in a pill or capsule that is swallowed.

A chemotherapy regimen (schedule) usually consists of a specific number of cycles given over a set period of time. A patient may receive 1 drug at a time or combinations of different drugs at the same 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 is any treatment that focuses on reducing symptoms, improving quality of life, and supporting patients and their families. Any person, regardless of age or type and stage of cancer, may receive palliative care. It works best when palliative care is started as early as needed in the cancer treatment process. 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, emotional support, and other therapies. You may also receive palliative treatments similar to those meant to eliminate the cancer, such as chemotherapy, surgery, or 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 palliative 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 can be addressed as quickly as possible. Learn more about palliative care.

Metastatic AdCC

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. Also, clinical trials might be an option. Learn more about getting a second opinion before starting treatment, so you are comfortable with your treatment plan chosen.

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 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.

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 does return 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.

When this occurs, a cycle of testing will begin again to learn as much as possible about the recurrence. After testing is done, you and your doctor will talk about your 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 also suggest clinical trials that are studying new ways to treat this type of recurrent cancer. Whichever treatment plan you choose, palliative care will be important for relieving symptoms and side effects.

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.

If treatment fails

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 advanced cancer 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 6 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 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. Or, use the menu to choose 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, 01/2016

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.

What are clinical trials?

Doctors and scientists are always looking for better ways to care for 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. In fact, every drug that is now approved by the U.S. Food and Drug Administration (FDA) was tested in clinical trials.

Many clinical trials focus on new treatments. Researchers want to learn if a new treatment is safe, effective, and possibly better than the treatment doctors use now. 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 can be some of the first to get a treatment before it is available to the public. However, there is no guarantee that the new treatment will be safe, effective, or better than what doctors use now.

Some clinical trials study new ways to relieve symptoms and side effects during treatment. Others study ways to manage the late effects that may happen a long time after treatment. Talk with your doctor about clinical trials for symptoms and side effects. There are also clinical trials studying ways to prevent cancer.

Deciding to join a clinical trial

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, in a clinical trial, they may receive no treatment by being given a placebo or a “sugar pill.” However, placebos are usually combined with standard treatment in most cancer clinical trials. 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.

Patient safety and informed consent

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.

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. 

Finding a clinical trial

Research through clinical trials is ongoing for all types of cancer. For specific topics being studied for AdCC, learn more in the Latest Research section.

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.

In addition, this website offers free access to a video-based educational program about cancer clinical trials, located outside of this guide.

The next section in this guide is Latest Research. It explains areas of scientific research currently going on for this type of cancer. Or, use the menu to choose 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, 01/2016

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.

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. 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. See below for more information on targeted therapies.

  • 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. Research is being done about the specifically looking into NOTCH1 gene mutations. These gene mutations may help doctors understand how the disease will respond to targeted therapy.  Learn more about targeted treatments.

  • Pathology for diagnosis. Doctors are looking at new ways to better diagnose this cancer. This will help doctors to provide more specific treatment plans based on what they learn. Researchers are looking at the rearrangement of the gene MYP as well as immunophenotyping testing for p63 and p40. These help to show whether a tumor is adenoid cystic carcinoma or basal cell adenocarcinoma.

  • 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.

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

Looking for More About the Latest Research?

If you would like additional information about the latest areas of research regarding AdCC cancer, explore these related items that take you outside of this guide:

The next section in this guide is Coping with Treatment. It offers some guidance in how to cope with the physical, emotional, and social changes that cancer and its treatment can bring. Or, use the menu to choose another section to continue reading this guide.  

Adenoid Cystic Carcinoma - Coping with Treatment

This section has been reviewed and approved by the Cancer.Net Editorial Board, 01/2016

ON THIS PAGE: You will learn more about coping with the physical, emotional, social, and financial effects of cancer and its treatment. This page includes several links outside of this guide to other sections of this website. To see other pages, use the menu.

Every cancer treatment can cause side effects or changes to your body and how you feel. For many reasons, people don’t experience the same side effects even when given the same treatment for the same type of cancer. This can make it hard to predict how you will feel during treatment.

As you prepare to start cancer treatment, it is normal to fear treatment-related side effects. It may help to know that your health care team will work to prevent and relieve side effects. Doctors call this part of cancer treatment “palliative care.” It is an important part of your treatment plan, regardless of your age or the stage of disease.

Coping with physical side effects

Common physical side effects from each treatment option for AdCC are described within the Treatment Options section. Learn more about side effects of cancer and its treatment, along with ways to prevent or control them. Changes to your physical health depend on several factors, including the cancer’s stage, the length and dose of treatment, you’re your general health.

Sometimes, physical side effects can last after treatment ends. Doctors call these long-term side effects. They call side effects that occur months or years after treatment late effects. Treating long-term side effects and late effects is an important part of survivorship care. Learn more by reading the Follow-up Care section of this guide or talking with your doctor.

Coping with emotional and social effects

You can have emotional and social effects as well as physical effects after a cancer diagnosis. This may include dealing with difficult emotions, such as anxiety or anger, or managing your stress level. Sometimes, patients have problems expressing how they feel to their loved ones, or people don’t know what to say in return.

Patients and their families are encouraged to share their feelings with a member of their health care team. You can also find coping strategies for emotional and social effects in a separate section of this website. This section includes many resources for finding support and information to meet your needs.

Coping with financial effects

Cancer treatment can be expensive. It is often a big source of stress and anxiety for people with cancer and their families. In addition to treatment costs, many people find they have extra, unplanned expenses related to their care. For some people, the high cost stops them from following or completing their cancer treatment plan. This can put their health at risk and may lead to higher costs in the future. Learn more about managing financial considerations, in a separate part of this website.

Caring for a loved one with cancer

Family members and friends often play an important role in taking care of a person with AdCC. This is called being a caregiver. Caregivers can provide physical, practical, and emotional support to the patient, even if they live far away.

Caregivers may have a range of responsibilities on a daily or as-needed basis. Below are some of the responsibilities caregivers take care of:

  • Providing support and encouragement

  • Giving medications

  • Helping manage symptoms and side effects

  • Coordinating medical appointments

  • Providing a ride to appointments

  • Assisting with meals

  • Helping with household chores

  • Handling insurance and billing issues

Learn more about caregiving.

Talking with your health care team about side effects

Before starting treatment, talk with your doctor about possible side effects. Ask:

  • Which side effects are most likely?

  • When are they are likely to happen?

  • What can we do to prevent or relieve them?

Be sure to tell your health care team about any side effects that happen during treatment and afterward, too. Tell them even if you don’t think the side effects are serious. This discussion should include physical, emotional, and social effects of cancer.

Also, ask how much care you may need at home and with daily tasks during and after treatment. This can help you make a caregiving plan.

The next section in this guide is Follow-up Care. It explains the importance of check-ups after cancer treatment is finished. Or, use the menu to choose another section to continue reading this guide.  

Adenoid Cystic Carcinoma - Follow-Up Care

This section has been reviewed and approved by the Cancer.Net Editorial Board, 01/2016

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

Care for people diagnosed with cancer doesn’t end when active treatment has finished. Your health care team will continue to check to make sure the cancer has not returned, manage any side effects, and monitor your overall health. This is called follow-up care.

Your follow-up care may include regular physical examinations, medical tests, or both. Doctors want to keep track of your recovery in the months and years ahead. 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.

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 if major surgery to the mouth or jaw has taken place during treatment. A prosthodontist is a dental specialist with additional training and certification in the restoration and replacement of broken teeth with crowns, bridges, and removable prosthetics, such as dentures.

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.

Learn more about the importance of follow-up care.

Watching for recurrence

One goal of follow-up care is to check for a recurrence. Cancer recurs because small areas of cancer cells may remain undetected in the body. Over time, these cells may increase in number until they show up on test results or cause signs or symptoms. During follow-up care, a doctor familiar with your medical history can give you personalized information about your risk of recurrence. Your doctor will also ask specific questions about your health. Some people may have blood tests or imaging tests as part of regular follow-up care, but testing recommendations depend on several factors including the type and stage of cancer originally diagnosed and the types of treatment given.

Managing long-term and late side effects

Most people expect to experience side effects when receiving treatment. However, it is often surprising to survivors that some side effects may linger beyond the treatment period. These are called long-term side effects. In addition, other side effects called late effects may develop months or even years afterwards. Long-term and late effects can include both physical and emotional changes.

Talk with your doctor about your risk of developing such side effects based on the type of cancer, your individual treatment plan, and your overall health. If you had a treatment known to cause specific late effects, you may also have certain physical examinations, scans, or blood tests to help find and manage them.

Keeping personal health records

You and your doctor should work together to develop a personalized follow-up care plan. Be sure to ask about any concerns you have about your future physical or emotional health. ASCO offers forms to help create a treatment summary to keep track of the cancer treatment you received and develop a survivorship care plan once treatment is completed.

This is also a good time to decide who will lead your follow-up care. Some survivors continue to see their oncologist, while others transition back to the general care of their family doctor or another health care professional. This decision depends on several factors, including the type and stage of cancer, side effects, health insurance rules, and your personal preferences.

If a doctor who was not directly involved in your cancer care will lead your follow-up care, be sure to share your cancer treatment summary and survivorship care plan forms with him or her, as well as all future health care providers. Details about your cancer treatment are very valuable to the health care professionals who will care for you throughout your lifetime.

The next section in this guide is Survivorship. It describes how to cope with challenges in everyday life after a cancer diagnosis. Or, use the menu to choose another section to continue reading this guide.  

Adenoid Cystic Carcinoma - Survivorship

This section has been reviewed and approved by the Cancer.Net Editorial Board, 01/2016

ON THIS PAGE: You will read about how to cope with challenges in everyday life after a cancer diagnosis. To see other pages, use the menu.

What is survivorship?

The word “survivorship” means different things to different people. Common definitions include:

  • Having no signs of cancer after finishing treatment.

  • Living with, through, and beyond cancer. According to this definition, cancer survivorship begins at diagnosis and includes people who continue to have treatment over the long term, to either reduce the risk of recurrence or to manage chronic disease.

Survivorship is one of the most complicated parts of having cancer. This is because it is different for everyone.

Survivors may experience a mixture of strong feelings, including joy, concern, relief, guilt, and fear. Some people say they appreciate life more after a cancer diagnosis and have gained a greater acceptance of themselves. Others become very anxious about their health and uncertain of how to cope with everyday life.

Survivors may feel some stress when frequent visits to the health care team end following treatment. Often, relationships built with the cancer care team provide a sense of security during treatment, and people miss this source of support. This may be especially true as new worries and challenges surface over time, such as any late effects of treatment, emotional challenges including fear of recurrence, sexuality and fertility concerns, and financial and workplace issues.

Every survivor has individual concerns and challenges. With any challenge, a good first step is being able to recognize your fears and talk about them. Effective coping requires:

  • Understanding the challenge you are facing,

  • Thinking through solutions,

  • Asking for and allowing the support of others, and

  • Feeling comfortable with the course of action you choose.

Many survivors find it helpful to join an in-person support group or an online community of survivors. This allows you to talk with people who have had similar first-hand experiences. Other options for finding support include talking with a friend or member of your health care team, individual counseling, or asking for assistance at the learning resource center of the center where you received treatment.

Changing role of caregivers

Family members and friends may also go through periods of transition. A caregiver plays a very important role in supporting a person diagnosed with cancer, providing physical, emotional, and practical care on a daily or as-needed basis. Many caregivers become focused on providing this support, especially if the treatment period lasts for many months or longer.

However, as treatment is completed, the caregiver's role often changes. Eventually, the need for caregiving related to the cancer diagnosis will become much less or come to an end. Caregivers can learn more about adjusting to life after caregiving in this article.

A new perspective on your health

For many people, survivorship serves as a strong motivator to make positive lifestyle changes.

People recovering from AdCC are encouraged to follow established guidelines for good health, such as not smoking, limiting alcohol, eating well, and managing stress. Regular physical activity can help rebuild your strength and energy level. Your health care team can help you create an appropriate exercise plan based upon your needs, physical abilities, and fitness level. Learn more about making healthy lifestyle choices.

In addition, it is important to have recommended medical check-ups and tests (see Follow-up Care) to take care of your health. Cancer rehabilitation may also be recommended, and this could mean any of a wide range of services such as physical therapy, career counseling, pain management, nutritional planning, and/or emotional counseling. The goal of rehabilitation is to help people regain control over many aspects of their lives and remain as independent and productive as possible.

Talk with your doctor to develop a survivorship care plan that is best for your needs.

Looking for More Survivorship Resources?

For more information about cancer survivorship, explore these related items. Please note these links will take you to other sections of Cancer.Net:

  • ASCO Answers Cancer Survivorship Guide: This 44-page booklet that helps people transition into life after treatment. It includes blank treatment summary and survivorship care plan forms. The booklet is available as a PDF, so it is easy to print out.

  • Cancer.Net Patient Education Video: View a short video led by an ASCO expert that provides information about what comes next after finishing treatment.

  • Survivorship Resources: Cancer.Net offers an entire area of this website with resources to help survivors, including for survivors in different age groups. 

The next section offers Questions to Ask the Doctor to help start conversations with your cancer care team. Or, use the menu to choose 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, 01/2016

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.

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.

Questions to ask after getting a diagnosis

  • 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, or laboratory test results, to me?

  • Did a pathologist with experience diagnosing salivary disease review my biopsy?

Questions to ask about choosing a treatment and managing side effects

  • What treatment plan do you recommend? Why?

  • What clinical trials are available for me? Where are they located, and how do I find out more about them?

  • 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 leading my overall treatment?

  • 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?

  • If I am worried about managing the costs of cancer care, who can help me?

  • 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?

Questions to ask about planning follow-up care

  • What is the chance that the cancer will come back? Should I watch for specific signs or symptoms?

  • What long-term side effects or late effects are possible based on the cancer treatment I received?

  • What follow-up tests will I need, and how often will I need them?

  • How do I get a treatment summary and survivorship care plan to keep in my personal records?

  • Who will be leading my follow-up care?

  • What survivorship support services are available to me? To my family?

The next section in this guide is Additional Resources. It offers some more resources on this website beyond this guide that may be helpful to you. Or, use the menu to choose 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, 01/2016

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.

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.

Beyond this guide, here are a few links to help you explore other parts of Cancer.Net:

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