What Is Adenoid Cystic Carcinoma?

Medically Reviewed by Elmer Huerta, MD, MPH and Zilpah Sheikh, MD on April 01, 2025
Written by Alyson Powell Key, Matt Smith
9 min read

Adenoid cystic carcinoma (ACC) is a rare kind of cancer. It usually starts in the glands that make your saliva. There are four glands under your tongue.

You have two submandibular and two sublingual glands on both sides of your jawbone. Submandibular means below your lower jaw (mandible). And sublingual means under your tongue (lingual). You also have one parotid gland on each side. Parotid means it's near your ear.

ACC can also show up in other parts of your mouth or throat. And it can spread to other areas of your body, like your sweat or tear glands. Doctors don't know what causes adenoid cystic carcinoma. But it may be linked to some carcinogens, such as pollution or asbestos.

"[ACC] differs from other head and neck cancers. They come from the lining inside our mouth, nose, and back of our throat.," explains Ansley Roche, MD, an assistant professor of surgery (otolaryngology) in the Head & Neck Cancers Multidisciplinary Clinic at Yale Cancer Center in New Haven, CT. "But [ACC] shows up in saliva glands."

Adenoid cystic carcinoma affects 4.5 out of every 100,000 people in the U.S. It happens a little more often in women than in men. Of all the salivary gland cancers, ACC is the second most common type. It's usually diagnosed between the ages of 40 and 60.

Adenoid cystic carcinoma tends to grow slowly. So, it can spread to other parts of your body before you notice any symptoms. ACC can also return to places where it's been treated before. It can also spread to your lungs, liver, or bones — where it's more serious.

The first sign of ACC may be a painless lump inside your mouth. It's typically under your tongue or inside your cheek. These lumps usually grow slowly and don't hurt. You might have some trouble swallowing, or your voice might sound hoarse. With ACC symptoms, you may have:

  • Muscle weakness
  • Trouble breathing through your nose (congestion)
  • Nosebleeds
  • Vision changes

This type of cancer can spread along your nerves, too. So, you might have some pain or numbness in your face. If you notice any of these symptoms, talk to your doctor. They'll help you figure out what may be going on.

After your doctor reviews your medical history, they'll examine your mouth. They'll look at your facial motion and how your jaw moves. Your doctor will ask about your past history of cancer. They'll also ask about pain and numbness, or if your lump has been getting bigger. 

Your doctor will measure the size of the lump in your mouth, too. They'll check out your scalp, skin, and neck. Your doctor will also look for any swollen lymph nodes near your tumor.

Lymph nodes are small bean-shaped parts to help your immune system fight off infection. Your doctor will look behind your ears and around your neck, too.

Imaging for adenoid cystic carcinoma

Your doctor may want to find out the size and place of your tumor, or if it's spread. To do this, you might have one of these tests:

  • MRI: Powerful magnets and radio waves make detailed images.
  • CT scan: Several X-rays taken from different angles can show more information.
  • PET scan: Radiation helps make three-dimensional color images.
  • Ultrasound: The use of high-intensity sound waves can show what's inside of your body.

CT and MRI can also help your doctor stage your cancer. The cancer stage tells you the type of cancer and how far it's spread. It also helps your doctor decide on the best treatments.

Biopsy for adenoid cystic carcinoma

 If your doctor thinks you might have adenoid cystic carcinoma, they'll often get a biopsy. A biopsy tests a small tissue sample of your tumor. Your doctor will either do a fine needle aspiration (FNA) or a core needle biopsy. They'll numb your skin first. FNA uses a small needle, and core needle biopsy uses a small cut.

A pathologist is a doctor who specializes in spotting diseases. They'll look at your tumor tissue under a microscope for signs of cancer. These kinds of tumors can take different forms. 

ACC can be solid, or round and hollow like a tube. Or it can be cribriform — have holes like Swiss cheese. The solid tumors usually grow faster.

Doctors don't know the exact cause of adenoid cystic carcinoma. But they believe it comes from changes to your genes over time.

"There are some genetic mutations that have been associated with adenoid cystic carcinoma, but they don't run in families," explains Dr. Roche. "It's a genetic mutation that just happens all of a sudden."

Your tumor fuses together two genes to make extra MYB protein. The protein helps your tumor grow. And higher protein levels show up in more than half of ACC tumors.

But these gene changes are not inherited. That means the changes to your DNA can happen during your lifetime. They can happen from chemicals in your environment, like tobacco or workplace chemicals.

Rarely, you can inherit genes that make you more sensitive to things like tobacco or chemicals. And it can increase your risk for certain cancers.

The usual treatment for adenoid cystic carcinoma is surgery. You'll likely get radiation therapy next. You could also have chemotherapy or targeted therapy.

Surgery for adenoid cystic carcinoma

Surgery will depend on where your tumor is located. It's most often in the parotid gland. But sometimes, it may be a submandibular or sublingual gland surgery. This type of surgery takes out the smaller salivary glands under your jaw or tongue. 

Most ACC starts on the outside of your gland. Your doctor will likely remove just the gland with cancer (a superficial parotidectomy). They'll avoid your facial nerve that runs through your parotid — to keep it healthy. 

When you have surgery, your doctor will check for cancer in other tissues, too. Unlike many other cancers that spread through your lymph nodes, ACC can move along your nerves. Your doctor will look closely at your nerves to check for cancer in the area. They'll take out any cancer and try to keep your nerves healthy.

Sometimes, they may need to take out your entire parotid gland (total parotidectomy). The surgery may also take out part of your windpipe or voice box. Part of a nerve may also need to be removed. That can mean you won't be able to move part of your face or it might droop. 

But your doctor may try to reconnect the damaged nerve with part of another one. "It doesn't fully restore movement, but it can restore tone," explains Dr. Roche. "So when you look at somebody, they don't have an obvious weakness." 

Another option is facial reanimation. In this, doctors implant muscle from another part of your body, typically the groin. 

"We use what's left of the facial nerve to power the muscle, so over time, patients can restore movement," says Dr. Roche.

Radiation for adenoid cystic carcinoma

If your doctor can't take out the entire tumor, radiation may be a good option. It's also a good treatment if your cancer has spread. Your doctor may suggest radiation if you're at higher risk or surgery isn't an option. 

Radiation can also help with your symptoms, like pain, bleeding, or trouble swallowing. External beam radiation therapy (EBRT) uses high-energy X-rays to destroy cancer cells. Here are a few ways your doctor uses EBRT:

  • Three-dimensional conformal radiation therapy uses computers to map where your tumor is at. It can shape and focus the exact dose for your tumor.
  • Intensity modulated radiation therapy also uses a computer to map your tumor. But it figures out how intense the radiation needs to be, too.
  • Proton beam radiation uses positively charged proton particle radiation, instead of X-rays. This therapy is still being studied.
  • Neutron therapy can target small tumors. It heats up tumor cells with 100-times more energy. Although it kills cancer cells, your normal cells recover. But neutron therapy is very limited.

Radiation therapy focused on your head and neck has side effects. During your radiation therapy, you may have:

  • Dry mouth

  • Difficulty swallowing

  • Soreness around the treated area

  • Damage to your teeth 

Check with your dentist and doctor to get help with the side effects.

Other therapies and clinical trials for ACC

A clinical trial may be a good option. It can depend on things like your cancer stage, place, and your risk factors. Talk to your doctor about trials in your area. Some trial medicines being studied include targeted therapies and immunotherapies.

Your doctor may suggest a targeted therapy, if you can't join a clinical trial. No immunotherapies, chemo medications, or targeted therapies are approved to treat ACC. But your doctor may use an "off-label" one. That means they'll prescribe one that's approved for another cancer. 

For example, sorafenib (Nexavar) is a vascular endothelial growth factor (VEGF) inhibitor some doctors use. It may stop your cancer cells from growing. It works for tumors that make too much VEGF protein.

Recent studies for ACC point out that it isn't easy to predict how the tumor will grow or if it'll come back. Experts think it's in part because ACC is so rare. It may also be because there are so many different gene changes causing your tumor.

It can be hard to get rid of the cancer entirely. Tumors may come back years later. They may be in the same place, or more likely somewhere new. ACC usually shows up in your lungs. How your medical condition progresses (prognosis) depends on your:

  • Place (location) of your tumor
  • Stage (grade) of your cancer (how big it is or if it's spread)
  • Perineural invasion (PNI) (spread of cancer along your nerves)

After your treatment, you'll need regular checkups to look for signs of new tumors. This can mean X-rays, CT scans, or MRIs. But it depends on your diagnosis.

Adenoid cystic carcinoma survival rate

Everyone is different and how your body responds to treatment matters. Here are a few survival numbers after ACC has been diagnosed:

  • About 80 in 100 people live at least 5 years.

  • About 61 in 100 people live at least 10 years.

If you've had treatment for your ACC, here are a few survival numbers: 

  • About 66 in 100 people live for 5 years if it's in your windpipe and voice box (larynx).

  • About 75 in 100 people live 10 years, if it starts in your breast. 

Remember, these numbers are estimates. They don't predict how long you'll live or respond to therapy. Talk to your doctor about your specific health and treatment plan.

Adenoid cystic carcinoma (ACC) is a rare cancer, usually starting in your salivary glands. But ACC also forms in other areas, like your mouth, throat, or tear and sweat glands. Experts don't know the exact cause but gene changes may drive its growth. Doctors usually treat ACC with surgery, followed by radiation therapy.

How rare is adenoid carcinoma?

Adenoid cystic carcinoma is rare. Less than 1% of head and neck cancers are ACC. Every year in the U.S., about 1,200 people get it.

Can you cure adenoid cystic carcinoma?

ACC often returns, in about 30% of cases. ACC can come back up to 20 years after treatment. It's also more likely to move or spread to other body parts.

ACC can be hard to treat because it can move along your nerves. Early diagnosis and treatment are important for a better outcome. If you have a lump in your mouth that isn't going away, see your dentist or doctor about it.

What is stage 4 ACC cancer?

Stage 4 (IV) ACC usually means the tumor is in nearby tissues. It may be in your jawbone, ear canal, or your facial nerves. Sometimes, the cancer cells are in your other organs, like the base of your skull or lungs. ACC rarely gets into your lymph nodes --- only about 5% to 10% of the time.