Adenoid Cystic Carcinoma (ACC)

What is adenoid cystic carcinoma?

Adenoid cystic carcinoma (ACC) is an uncommon type of cancer that usually develops in your salivary glands or other areas of your head and neck. Sometimes, it can form in other parts of your body, including your skin, breast tissue, cervix or prostate gland. ACC tumors might be solid, round and hollow. Or they might have holes in them (like Swiss cheese).

ACC tumors grow slowly. But they can become aggressive and unpredictable over time. That means that even if surgery gets rid of the cancer, it could come back later. Most people with adenoid cystic carcinoma will have recurrence (the cancer will come back) within five to 10 years. If you receive a diagnosis, you’ll need routine scans and checkups to monitor your condition.

How common is adenoid cystic carcinoma?

Adenoid cystic carcinoma is rare. It makes up about 1% of all head and neck cancers and affects 4.5 out of every 100,000 people in the general population. In the United States, about 1,200 people receive a diagnosis every year.

Most people with ACC are between the ages of 40 and 60. The condition is slightly more common in people assigned female at birth (AFAB).

What are the symptoms of adenoid cystic carcinoma?

Adenoid cystic carcinoma symptoms depend on the size and location of the tumor. You might have:

• A painless lump.
• Difficulty breathing.
• Difficulty swallowing.
• Hoarseness.
• Muscle weakness.
• Nasal congestion.
• Nosebleeds.
• Pain.
• Vision changes.

Because ACC is slow-growing, it doesn’t always cause symptoms.

What causes adenoid cystic carcinoma?

Experts aren’t sure what causes adenoid cystic carcinoma. They think it happens because of genetic mutations (changes) that occur over time. ACC doesn’t run in families and there are no known risk factors.

What are the complications of adenoid cystic carcinoma?

Adenoid cystic carcinoma can spread (metastasize) in the following two ways:

• Perineural invasion. In most cases of ACC, cancer cells creep into the nerve fibers around the tumor. It happens on such a microscopic level that it can be hard for healthcare providers to detect on imaging scans or during surgery.
• Through your bloodstream. Cancer cells from the tumor can break off and travel to other areas of your body via your bloodstream.

Metastatic (or Stage 4) adenoid cystic carcinoma most commonly spreads to your lungs and liver. Unlike other types of cancer, ACC doesn’t spread to lymph nodes very often. But it happens in about 5% to 10% of cases.

How is adenoid cystic carcinoma diagnosed?

If your healthcare provider thinks you may have adenoid cystic carcinoma, they will likely take a biopsy. They’ll send your tissue sample to a pathologist for testing. Your provider may also take one or more imaging tests, like:

• CT scans (computed tomography scans).
• MRI (magnetic resonance imaging).
• PET scans (positron-emission tomography scans).
• Ultrasound.

Due to the rarity of ACC, it can take some time to diagnose. You might need several rounds of testing before you receive a diagnosis and begin treatment.

How is adenoid cystic carcinoma treated?

Adenoid cystic carcinoma treatment depends on the location of the tumor and whether it has spread. Options include:

• Surgery. Surgery is the go-to treatment for adenoid cystic carcinoma. The goal is to remove the entire tumor, if possible. In some cases, your surgeon may have to remove lymph nodes or part of a nerve.
• Radiation therapy. This can get rid of any remaining cancer cells after surgery. Or you might have radiation therapy as a stand-alone treatment.
• Chemotherapy. Drugs like cisplatin, vinorelbine, paclitaxel and carboplatin can treat advanced or metastatic ACC.
• Targeted therapy. Drugs like lenvatinib and sunitinib block the actions of proteins that encourage cancer growth. Healthcare providers use targeted therapy for advanced or metastatic cases of ACC.

Can adenoid cystic carcinoma be prevented?

Because there are no identifiable risk factors for ACC, there’s no known way to reduce your risk. There’s also no known way to prevent ACC from coming back.

What’s the outlook for adenoid cystic carcinoma?

Adenoid cystic carcinoma is a slow-growing cancer. Healthcare providers can often detect and treat it early with successful results. But it’s common for ACC to recur (return) many years later — either in the same place or a different area of your body. When this happens, it’s more difficult to treat.

Survival rates

The overall five-year survival rate for people with adenoid cystic carcinoma is 80.4%. This means that 80.4% of people with this condition are still alive five years later. The 10-year survival rate is 61.3%.

Remember, survival rates are only estimates. They can’t tell you how long you’ll live or how you’ll respond to treatment. To learn more about survival rates and what they mean for you, talk to your healthcare provider.

How can I take care of myself?

Because ACC is so rare, it can take a while to get a diagnosis. This can take a toll on your mental and emotional health. Talking with a counselor or therapist can help you process complicated feelings. Many people find solace in support groups, or by taking music or art therapy classes. Try reducing stress through meditation or other mindfulness exercises.

When should I see my healthcare provider?

If you have adenoid cystic carcinoma, tell your healthcare provider any time you develop new or worsening symptoms. A change in symptoms could mean that your tumor has grown.

What questions should I ask my doctor?

Here are some questions you might consider asking your healthcare provider about ACC:

• Where’s the tumor located?
• What symptoms might I develop?
• Has cancer spread anywhere else in my body?
• Will you be able to remove the tumor with surgery?
• Will I need radiation therapy?
• Can I work while undergoing cancer treatment?
• How long will treatment take?