Adenoid Cystic Carcinoma (ACC)

Overview

What is adenoid cystic carcinoma?

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).

Who does adenoid cystic carcinoma affect?

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.

How common is adenoid cystic carcinoma?

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.

Symptoms and Causes

What are the symptoms of 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.

ACC of the salivary gland

Symptoms may include:

  • Facial pain.
  • Facial drooping.
  • Numbness in your lip or other areas of your face.

ACC of the lacrimal gland

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:

  • Vision changes.
  • Proptosis (bulging eye).
  • Pain, swelling.

ACC of the skin

When adenoid cystic carcinoma forms in the skin, it most commonly affects the scalp or the external ear canal. Symptoms may include:

  • Pain.
  • Pus.
  • Bleeding.
  • Increased sensitivity.
  • Hair loss in the affected area.

ACC of the lower respiratory tract

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:

  • Difficulty breathing.
  • Stridor (a high-pitched, wheezing sound when breathing in).
  • Hoarseness.

ACC of the larynx

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:

ACC of the esophagus

Adenoid cystic carcinoma can also develop in your esophagus, though it’s rare. When this occurs, you may have:

  • Difficulty swallowing liquids, soft foods and even saliva.
  • Regurgitation of liquids and food.
  • Weight loss associated with the inability to keep foods and liquids down.

ACC of the breast

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).

ACC of the cervix

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:

ACC of the prostate

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.

What causes adenoid cystic carcinoma?

Experts aren’t sure what causes adenoid cystic carcinoma. There are currently no known risk factors.

How does adenoid cystic carcinoma spread?

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.

Diagnosis and Tests

How is adenoid cystic carcinoma diagnosed?

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:

  • A CT (computed tomography) scan. A CT scan captures numerous X-ray images and stitches them together for a detailed, 3-D picture.
  • Magnetic resonance imaging (MRI). This procedure uses powerful magnets and radio waves to capture detailed images inside your body.
  • A PET scan. This imaging test uses a safe injectable chemical (called a radiotracer) to reveal how your organs are functioning in real time.

Management and Treatment

How is adenoid cystic carcinoma treated?

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.

Side effects of surgery for ACC

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.

Side effects of radiation treatment for ACC

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.

Is adenoid cystic carcinoma curable?

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.

Prevention

How can I reduce my risk for adenoid cystic carcinoma?

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.

Outlook / Prognosis

What can I expect if I have adenoid cystic carcinoma?

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.

Is adenoid cystic carcinoma fatal?

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.

Living With

When should I see my 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.

What questions should I ask my healthcare provider?

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:

  • Where is my tumor located?
  • What symptoms are possible?
  • 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?

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.

References

  • Ammad Ud Din M, Shaikh H. Adenoid Cystic Cancer. (https://www.ncbi.nlm.nih.gov/books/NBK557855/) [Updated 2021 Jun 20]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2021 Jan-. Accessed 12/15/21.
  • Cancer.Net. Adenoid Cystic Carcinoma. (https://www.cancer.net/cancer-types/adenoid-cystic-carcinoma/view-all) Accessed 12/15/21.
  • National Organization for Rare Disorders. Adenoid Cystic Carcinoma. (https://rarediseases.org/rare-diseases/adenoid-cystic-carcinoma/) Accessed 12/15/21.
  • Ouyang DQ, Liang LZ, Zheng GS, Ke ZF, Weng DS, Yang WF, Su YX, Liao GQ. Risk factors and prognosis for salivary gland adenoid cystic carcinoma in southern china: A 25-year retrospective study. (https://pubmed.ncbi.nlm.nih.gov/28151884/) Medicine (Baltimore). 2017 Feb;96(5):e5964. doi: 10.1097/MD.0000000000005964. PMID: 28151884; PMCID: PMC5293447. Accessed 12/15/21.

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