Adenoid cystic carcinoma (ACC) is a rare type of cancer that typically starts in the saliva glands of the mouth or other areas of the neck and head. It may not cause symptoms in the early stages, but they can develop as the tumor grows.

Although ACC often appears in the saliva glands of the mouth, it can develop in any secretory glands throughout the body. Its distinctive shape helps doctors identify it when it occurs.

Read on to learn about the symptoms and causes of ACC. This article also explores its diagnosis, treatment options, and more.

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A person may not experience any symptoms from ACC when it first starts. It is slow growing and may develop over several years.

When symptoms occur, they can vary according to the location of the tumor.

Tumors in the salivary gland can cause symptoms such as:

  • facial numbness
  • a painless lump in the mouth, face, or neck
  • drooping in the face
  • trouble swallowing or opening the mouth

If the tumor starts in the breasts, a person may notice a slow-growing lump that may be painful or tender to the touch.

When a tumor develops in the lacrimal glands around the eyes, the individual may develop a bulging eye or changes in their vision. The lacrimal glands are responsible for producing tears.

Tumors that appear in the skin may be painful. They may create bloody or pus discharge and may be sensitive to touch.

If the tumor develops in the larynx and trachea, a person may experience issues with breathing or speech or notice hoarseness. The larynx and trachea are also known as the voice box and windpipe.

ACC develops due to mutations in the secretory glands. Researchers do not exactly know what causes these mutations to occur.

Some experts suspect it may happen due to changes to the genes that occur during a person’s lifetime. This is different from inherited conditions, where someone is born with changes to their genes.

Some studies indicate that changes to the p53 tumor suppressor gene may result in more aggressive cases of ACC.

As part of the diagnosis, a doctor will likely also perform a physical examination and review personal and family medical history.

Doctors may be able to identify ACC tumors due to their unique shape and through further examination under a microscope. However, they need to differentiate between ACC and other conditions that may affect the salivary glands or other areas of the body, as they can share some similar features.

Examining a biopsy sample under a microscope can help determine if the structure is cancerous. A biopsy involves removing part of the tumor and then sending it to a laboratory for analysis.

The doctor may also order a scan, such as:

  • X-rays
  • CT scans
  • MRIs
  • PET scans

Learn about what to expect from a biopsy.

Treatment for ACC can differ for each person. Factors that influence treatment can include:

  • age
  • location of the tumor
  • whether or not the cancer has spread
  • a person’s overall health
  • personal preferences

Treatments for ACC may involve one or more of the following:

  • surgery, which involves removing the tumor
  • chemotherapy, which uses medication to destroy cancer cells
  • radiation therapy, which uses high-energy rays to destroy cancer cells to help shrink the tumor

Surgery is the standard therapy for ACC. If surgical procedures are unsuitable, a doctor may recommend another treatment or combination of methods.

ACC is a rare form of cancer. Doctors diagnose about 1,200 new cases of the disease annually in the United States.

Of these, experts estimate that:

  • 58% have oral cavity, salivary glands, or pharynx tumors
  • 17% have respiratory system tumors
  • 12% develop breast tumors
  • 13% develop tumors in other areas of the body, such as the prostate or skin

ACC also accounts for about 10% of all head and neck cancers. The disease is most common in people between the ages of 40 and 60 years.

Complications can develop with ACC but may depend on the tumor’s location.

Some possible complications can include:

  • hair loss if it develops near the scalp
  • vision changes
  • weakness or numbness in the face
  • trouble breathing
  • changes in voice

ACC can also spread to local lymph nodes or nerves. It can also metastasize — or spread — to distant areas of the body. If this occurs, it often ends up in the lungs or liver.

Since experts do not know the exact causes of ACC, it may not be possible to prevent the disease.

It is best for a person to contact a doctor if they have concerns about ACC.

Most people who develop ACC live for at least 5 years. However, most will also develop a recurrence of the cancer.

One study of 160 individuals with ACC found that the 5-year survival rate was 89%, while the 15-year survival rate was 40%.

Survival estimates do not guarantee whether someone will or will not be alive in 5 years. Instead, they show how many people have survived following a cancer diagnosis. They are useful for doctors to help explain a person’s overall outlook, but several other factors specific to the individual will factor in their overall survival.

The survival rate refers to the proportion of people who are still alive for a length of time after receiving a particular diagnosis. For example, a 5-year survival rate of 50% means that 50%, or half, of the people are still alive 5 years after receiving the diagnosis.

It is important to remember that these figures are estimates and derive from the results of previous studies or treatments. A person can consult a healthcare professional about how their condition will affect them.

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Adenoid cystic carcinoma (ACC) is a rare, slow-growing cancer that starts in the secretary glands. It often develops in the salivary glands in the mouth but can also develop in other areas of the body.

Symptoms depend on the location of the tumor. Its exact cause is still unclear, but scientists suspect genetic changes during a person’s life cause its development.

Diagnosis often involves the use of imaging tests and a biopsy. Surgery is the most common treatment, but a doctor may also recommend other treatments such as chemotherapy or radiation therapy.

It is best to contact a doctor as soon as there are concerns about ACC. The doctor can order tests to confirm the diagnosis and advise on suitable treatments.