Adenoid cystic carcinoma (ACC) is a rare type of cancer that typically develops in your salivary glands or other parts of your head or neck. It may also develop in other areas, such as your breast, lungs, or skin.
About 1,224 people receive a diagnosis of ACC in the United States each year. It’s one of the most common salivary gland cancers but is considered a rare cancer overall.
ACC tends to develop slowly, but there’s a high chance that it will return after you receive treatment. Doctors usually recommend regular monitoring for the rest of your life even if the cancer is cured.
Researchers are continuing to examine the best way to treat ACC. It’s likely that survival rates will continue to improve as they improve their understanding.
In this article, we cover everything you need to know about ACC including symptoms, how it’s treated, and the outlook of people with this cancer.
Other names for adenoid cystic carcinoma
ACC is also called cribriform carcinoma or cylindroma. It’s classified as a type of adenocarcinoma. Adenocarcinoma is a type of cancer that develops in glandular cells.
ACC symptoms depend on which part of your body it grows in. The
If ACC develops in your salivary glands, it might eventually cause symptoms such as:
- numbness of your lower lip or other facial areas
- nerve impingement causing:
- weakness in facial muscles
- pain
- lump on the roof of your mouth, under your tongue, or in the bottom of your mouth
- difficulty swallowing
- hoarseness
- dull pain
Here’s a look at some of the other potential symptoms depending on where the cancer develops.
| Location | Symptoms |
|---|---|
| Tear glands | – bulging eye – changes in your vision |
| Skin, primarily scalp or ear | – pain – discharge of pus or blood – reddish nodule or plaque – hair loss if it develops on your scalp |
| Respiratory tract | – trouble breathing – hoarseness – high-pitched sound when breathing – general malaise – weight loss – pain – recurrent lung inflammation – coughing up blood |
| Voicebox | – trouble breathing – shortness of breath – hoarseness – changes in speech – trouble swallowing – sore throat – visible mass in neck |
| Esophagus | – trouble swallowing – regurgitating food or liquids – weight loss |
| Breast | – tender or painful mass in one breast, usually around the nipple – indentation of the nipple |
| Cervix | – vaginal bleeding – watery or bloody discharge |
| Prostate | – poor urine flow – frequent urination – trouble starting urine stream |
Researchers don’t know why ACC forms in some people. Because this cancer grows so slowly and is so rare, developing cell lines to study has been difficult. ACC also appears to behave differently depending on its location in the body.
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According to the Adenoid Cystic Carcinoma Research Foundation, about 60% of people with ACC are women. It most often develops in people between the ages of 40 to 60, and about half of people are over the age of 58 when they get a diagnosis.
ACC hasn’t been found to be associated with:
- smoking
- drinking alcohol
- infections
- your ethnicity
- family history
A doctor will start the diagnostic process with a physical exam and by looking at your personal and family medical histories. They may order imaging or a biopsy if they suspect you could have cancer.
A biopsy is the primary test used to make the diagnosis. It involves extracting a small tissue sample so that the cells can be analyzed in a laboratory. Depending on the location of the tumor, the biopsy may be taken surgically by removing part of the tumor or with a long thin needle.
Imaging can help your doctor determine the size and extent of the tumor. They may order:
- magnetic resonance imaging (MRI)
- computed tomography (CT) scans
- positron emission tomography (PET) scans
Other location-specific diagnostic tests
Your doctor may order a number of other specific diagnostic tests depending on where the cancer is located. These might include:
| Location | Additional tests |
|---|---|
| Lungs | – insertion of a tube through your nose or mouth to take images of your lower respiratory tract |
| Larynx | – laryngoscopy – examination with a mirror |
| Esophagus | – barium X-ray procedure – endoscopy |
| Breast cancer | – mammogram |
| Cervix | – pap smear – colposcopy |
| Prostate | – blood tests – pyelography – ultrasound |
The most common treatments for ACC are surgery and radiation therapy. The Food and Drug Administration (FDA) hasn’t approved chemotherapy yet for treating ACC, but some people may receive chemotherapy for advanced cancer.
Surgery and radiation therapy
A surgeon may be able to remove your tumor depending on where it develops. The American Society of Clinical Oncology strongly recommends that all people treated surgically should also be offered radiation therapy to destroy any cancer cells not fully removed.
If surgery isn’t possible, you may receive radiation therapy alone.
About half of people are cured with a combination of radiation and surgery.
Other treatments
Chemotherapy generally isn’t used for ACC because this type of cancer seems to be less responsive than many other cancers.
Chemotherapy and another type of drug therapy called targeted therapy are under investigation in clinical trials.
In a review published in January 2023, researchers found that the drugs that show the most promising results are:
Your doctor may recommend taking part in a clinical trial of one of these treatments if you have advanced cancer.
ACC usually grows slowly, but it tends to be difficult to treat.
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- 5-year survival rate: 80.4%
- 10-year survival rate: 61.3%
- 15-year survival rate: 29.4%
You’ll likely need to be monitored for the rest of your life even if your treatment is successful. About
- more advanced cancer
- increased age
- involvement of your skull base
- relapse within 3 years
- cancer found at the edges of a surgically removed tumor
- cancer in the space surrounding nerves
- spread to your vertebrae
What are 5-year, 10-year, and 15-year survival rates?
Health professionals often use survival rates as a measure of someone’s outlook. It refers to the percentage of people with the disease who are still alive at least 5, 10, or 15 years after their diagnosis.
Relative survival rates are another commonly used term. This is a measure of how many people with the disease are alive 5 years later compared with people without the disease.
ACC is a cancer that most often develops in your salivary glands or other parts of your head or neck. It can also develop in other body parts such as your lungs, breast, or skin.
ACC is most often treated with surgery and radiation therapy.
Researchers are investigating some medications that may help treat advanced ACC. Your doctor can help you figure out the best treatment option for you and let you know if you may be eligible for any clinical trials.