Adenoid cystic carcinoma (ACC) is a rare type of cancer that forms in the salivary glands. It can also affect your throat, mouth or other parts of your body, including your tear or sweat glands. While ACC is slow-growing with a high five-year survival rate, it often comes back after many years. Survival rates drop at the 10 year mark.
Adenoid cystic carcinoma (ACC) is an uncommon type of cancer that usually develops in the salivary glands or other areas of the head and neck. Sometimes it can form in other parts of the body, including your skin, breast tissue, cervix or prostate gland. ACC tumors may be solid, round and hollow or they may have holes in them (like Swiss cheese).
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Most people with ACC are between the ages of 40 and 60. The condition is slightly more common in women than men (60% vs 40%). This is because ACC occurs more in female-specific sites (such as the breast and cervix) compared to male-specific sites.
Adenoid cystic carcinoma is considered rare. Statistically, out of the 500,000 people who are diagnosed with cancer each year, approximately 1,200 of them have adenoid cystic carcinoma.
Adenoid cystic carcinoma can affect several different body parts and systems. ACC symptoms vary significantly depending on the area of your body that’s affected.
Symptoms may include:
Some people develop adenoid cystic carcinoma in the gland that produces tears. (Note: Most cases occur in adults, but some have been diagnosed in children. ACC is usually less aggressive when it develops in young people.) Possible symptoms include:
When adenoid cystic carcinoma forms in the skin, it most commonly affects the scalp or the external ear canal. Symptoms may include:
Adenoid cystic carcinoma of the lower respiratory tract usually forms in the mucous glands of your trachea. This can cause gradual blockage of your windpipe, resulting in:
ACC tumors of the larynx (voice box) can affect the area directly below the glottis — the slit-like opening between the vocal cords. People with this condition may have a range of symptoms, including:
Adenoid cystic carcinoma can also develop in your esophagus, though it’s rare. When this occurs, you may have:
Unlike other forms of breast cancer, ACC of the breast is usually less aggressive. In most cases, it’s not likely to spread. People with this type of adenoid cystic carcinoma typically develop a moveable mass in one breast, usually near the areola (the dark area of skin around your nipple).
People can develop adenoid cystic carcinoma of the cervix, usually after menopause. It’s very aggressive and is characterized by a large cervical mass. Symptoms may include:
When ACC forms in the prostate, it’s considered a subtype of prostate adenocarcinoma. Potential symptoms include:
Adenoid cystic carcinoma can also affect other areas of your body, though these cases are rarer.
Experts aren’t sure what causes adenoid cystic carcinoma. There are currently no known risk factors.
Adenoid cystic carcinoma usually spreads along nerves or through the bloodstream. It spreads to lymph nodes in approximately 5% to 10% of cases. When ACC metastasizes (spreads) beyond the lymph nodes, it most commonly spreads to your lungs. In most cases, lung metastases grow very slowly over months and even years.
Local recurrence (return of cancer) is more common than distant metastasis. This happens when cancer spreads along the nerves of your face. It can be aggressive and difficult to treat.
If your healthcare provider thinks you may have adenoid cystic carcinoma, they will likely take a biopsy. The tissue sample will be sent to a pathology lab for further testing. Your provider may also take one or more imaging tests, including:
In most cases, adenoid cystic carcinoma is treated first with surgery and may be followed with radiation therapy in some cases. Your surgeon removes the tumor and some healthy tissue surrounding it. They’ll also check to ensure that cancer hasn’t spread anywhere else. In some cases, part of a nerve may need to be removed. In some cases, lymph nodes in the neck may also be surgically removed.
If your surgeon can’t remove the entire tumor without harming other organs, or if there are other high-risk features of the tumor such as large size or nerve or lymph node involvement, then radiation therapy may be recommended. Systemic therapy, including chemotherapy and other targeted agents, may be used in cases of recurrent disease that is no longer amenable to surgery or RT, or in widely metastatic disease.
One potential side effect of surgery is facial drooping. This occurs if your facial nerve is cut, which may be necessary in some cases. Permanent facial drooping is most common with ACC of the parotid gland.
Reconstructive surgery may be an option to improve facial drooping. Ask your healthcare provider about what to expect in your situation and how to prepare for these side effects.
Radiation therapy side effects include fatigue and skin changes, such as dryness, peeling or itching. However, there are specific side effects related to radiation therapy in the head and neck area:
If you’re undergoing radiation therapy in the head and neck area, it’s important to visit your dentist regularly. They can help keep your oral health in check during your cancer treatment.
Yes. The most common types of adenoid cystic carcinoma are slow growing. As a result, many forms of ACC are diagnosed early and treated successfully. It’s important to know, however, that ACC can potentially come back — often many years after your initial treatment.
Because there are no identifiable risk factors for adenoid cystic carcinoma, there is currently no known way to reduce your risk of the disease. There is also no known way to prevent ACC from coming back.
Adenoid cystic carcinoma is a slow growing cancer. For this reason, many cases are detected early and the first round of treatment is often successful. However, it’s common for cancer to return many years later.
ACC can be considered a life-limiting disease in the event it recurs or metastasizes. While most people respond well to their initial treatment of surgery (with or without radiation), cancer tends to come back later on — either in the same place or a different area of the body.
The overall five-year survival rate for all people with adenoid cystic carcinoma is 89%. This means that 89% of people who are diagnosed with the condition are still alive five years later. The estimated 10-year survival rate is less than 70%. Keep in mind that survival rates are only estimates. They can’t tell you how long you’ll live or how you’ll respond to your personalized treatment plan. To learn more about survival rates, talk to your healthcare provider.
If you’ve been diagnosed with adenoid cystic carcinoma, it’s important to call your healthcare provider anytime you develop new symptoms or existing symptoms worsen. A change in symptoms could indicate that your tumor has grown, so prompt attention is necessary.
It’s important to learn all you can about your condition so you can make informed decisions about your treatment plan and overall health. Here are some questions you might consider asking your healthcare provider:
A note from Cleveland Clinic
An ACC diagnosis can be shocking, leading to feelings of anger, sadness and frustration. If you or a loved one has been diagnosed with this disease, ask your healthcare provider about the many resources available. You may also want to join a local support group, which can be extremely beneficial for your mental and emotional health.
Last reviewed by a Cleveland Clinic medical professional on 12/15/2021.
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