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Adenoid Cystic Carcinoma

Adenoid cystic carcinoma (ACC) is a rare type of cancer that forms in glandular tissues, most common in the head and neck, but it can also begin in other areas.  

ACC often starts in the salivary glands in the neck, mouth or throat, which make saliva. Saliva keeps the mouth moist, helps you swallow food and protects the mouth against infections. 

Malignant (cancerous) tumours can spread to other parts of the body through the bloodstream or lymph vessels and form another tumour at a new site. This new tumour is known as secondary cancer or metastasis. 

ACC is considered rare. Around 330 Australians are diagnosed with a salivary gland cancer each year. ACC can develop at any age but is more common in people aged 40 to 60 years.  

Treatment

The main treatments for ACC include surgery and radiation therapy.  

Surgery

Surgery is usually the most effective treatment for ACC if the cancer can be safely removed. Surgery usually involves removing the tumour and some healthy tissue around the tumour, and this is called a wide local excision. The healthy tissue is removed to help reduce the risk of the cancer coming back in that area. 

The surgeon will examine nearby nerves and lymph nodes and may remove them if they are involved. It can be a difficult decision to remove nerves, especially major nerves or those that control the face. 

Radiation therapy (radiotherapy)

Radiation therapy uses high energy rays to destroy cancer cells. It may be used effectively for ACC after surgery to destroy any remaining cancer cells and stop the cancer from returning.  

Radiotherapy might also be used alone if surgery is not possible, for example:  

  • if the cancer is in a place in the body that is too hard to reach, using surgery  
  • if the cancer has spread to other parts of the body (e.g. palliative radiation for pain management). 

Chemotherapy

Chemotherapy is the use of drugs to kill or slow the growth of cancer cells. You may have one chemotherapy drug or a combination of drugs. 

Chemotherapy is not commonly used for ACC. It may be considered when the cancer is advanced as part of palliative treatment or a clinical trial. Your medical oncologist will discuss options with you. 

Palliative care

In some cases of ACC, your medical team may talk to you about palliative care. Palliative care aims to improve your quality of life by alleviating symptoms of cancer. 

As well as slowing the spread of ACC, palliative treatment can relieve pain and help manage other symptoms. Treatment may include radiotherapy, chemotherapy or other drug therapies. 

Risk factors

The cause of ACC is not known. It might develop from genetic changes during a person’s lifetime, rather than inheriting a faulty gene. ACC is not considered to be hereditary. 

Early symptoms

ACC generally develops slowly, sometimes over several years, and may not cause symptoms. It is often diagnosed as a single tumour but may have spread to nearby lymph nodes by the time it is diagnosed in a small number of cases.  

Rarely, ACC can spread along nerves or metastasise to other parts of the body (usually the lungs, liver or bone) and cause problems. It can also be unpredictable, growing slowly for some time and then suddenly growing quickly. ACC may behave differently in different people.  

The initial symptoms of ACC depend on the location of the tumour. Early lesions of the salivary glands may appear as painless, usually slow-growing masses underneath the normal lining of the mouth or skin of the face. Because there are many salivary glands under the mucosal lining of the mouth, throat, and sinuses, lumps in these locations could be from this type of tumour. Symptoms may include:  

  • Painless lump in the mouth, face or neck.
  • Numbness in the face, weakness in facial muscles (drooping in the face).
  • Difficulty swallowing or opening mouth.
  • An abnormal area on the lining of the mouth.
  • A bump or nodule in front of the ear or underneath the jaw.
  • Bulging eye or changes in vision.
  • Voice hoarseness, changes in speech or difficulty breathing.
  • Pain on your skin, increased sensitivity; pus and/or blood discharge or dull pain where the tumour may be.
  • A slow growing lump in your breast that may be tender or cause pain. 

Not everyone with the symptoms above will have cancer but see your general practitioner (GP) if you are concerned. 

Diagnosis/diagnosing

If your doctor suspects that you may have ACC, they will take your medical history, perform a physical examination (including feeling for any lumps) and carry out specific tests depending on the location of the suspected tumour.  

If the results suggest that you may have ACC, your doctor will refer you to a specialist for more tests and scans. The below tests and procedures may be used.

CT scan

Special machines are used to scan and create pictures of the inside of your body. You may have an injection of dye into your veins before the scan which makes the pictures clearer.  During the scan, you will lie on a table that moves in and out of the scanner.  A CT scan takes about 10-30 minutes.  

MRI

An MRI scan produces detailed cross-sectional pictures of your body and can show the extent of any tumours.  You will lie on a table that slides into a large metal tube open at both ends. An MRI scan takes about 30-90 minutes.  

PET scan

Before having the PET scan, you will be injected with a small amount of radioactive solutions. You will need to sit for 30-90 minutes so the solution can move around your body.  Many cancer cells will show up brighter on the scan. The scan takes around 30 minutes.  

Ultrasound

Soundwaves are used to create pictures of the inside of your body. You will lie down and a gel will be spread over the affected part of your body , and then a small device (transducer) is moved over the area. The ultrasound takes about 15 minutes and is painless.  

Biopsy

If your doctor sees any abnormal or unusual-looking areas, they may remove a small sample of the tissue for closer examination, a biopsy. A pathologist will look at the sample under a microscope to check for signs of disease or cancer.  

Prognosis (Certain factors affect the prognosis and treatment options)

The 5-year survival rate for people with ACC is approximately 89%. The 15-year survival rate for people with ACC is approximately 40%.

A late recurrence of ACC is common and can occur many years after initial treatment. A recurrence is most commonly diagnosed at the metastatic stage, meaning it has spread to other parts of the body. Tumour growth for ACC is often slow, and people may live a long time with metastatic disease. 

References

Some references are to overseas websites. There may be references to drugs and clinical trials that are not available here in Australia.