What Is Adenoid Cystic Carcinoma? Everything to Know

Medically Reviewed By Faith Selchick, DNP, AOCNP
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Adenoid cystic carcinoma is a rare type of adenocarcinoma. It is a type of cancer that typically originates in the salivary glands. Adenoid cystic carcinoma can also occur in other areas of the head, neck, and other parts of the body. This includes:

  • breasts
  • skin
  • cervix
  • prostate gland

Symptoms can depend on the size and location of the carcinoma. When it affects the salivary glands, common symptoms include facial numbness, weakness, and pain.

Read on to find out more about the symptoms of adenoid cystic carcinoma. This guide also includes causes, treatments, diagnosis, and more information.

What are the symptoms of adenoid cystic carcinoma?

There is a closeup of a person having their face examined.
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Adenoid cystic carcinoma may develop slowly over several years. In some cases, you may not experience any symptoms.

Symptoms that may occur can depend on the location and size of the tumor.

Salivary gland symptoms

If adenoid cystic carcinoma grows in the salivary gland, you may experience symptoms including:

Lacrimal gland symptoms

The lacrimal gland is responsible for producing tears. If the tumor grows in the lacrimal gland, your symptoms may include:

  • pain
  • changes in your vision
  • a bulging eye

Larynx and trachea symptoms

You may know the larynx and trachea as the voice box and windpipe, respectively. If the tumor grows here, symptoms can include:

Skin symptoms

Adenoid cystic carcinoma can begin in the skin. Skin symptoms can include:

  • increased sensitivity
  • pain
  • pus
  • bloody discharge

Breast symptoms

An adenoid cystic carcinoma tumor can also begin in the breast. When this happens, you may experience a tender or painful lump that grows slowly.

Adenoid cystic carcinoma is a type of adenocarcinoma. Learn more about the types and symptoms of adenocarcinoma.

What causes adenoid cystic carcinoma?

Researchers do not know the exact cause of adenoid cystic carcinoma.

However, genetic changes or mutations may be responsible for the development of the condition. In particular, research focuses on a protein resulting from the MYB-NFIB gene fusion. The fusion protein is present in around 90–95% of adenoid cystic carcinoma tumors.

What are the treatments for adenoid cystic carcinoma?

You will likely require surgery for adenoid cystic carcinoma. Your surgeon will remove the tumor and affected tissue.

Your doctor may recommend radiation therapy to destroy any remaining cancer cells after surgery or as an alternative. Your doctor will be able to advise on whether or not surgical removal is possible.

Chemotherapy does not effectively treat adenoid cystic carcinoma.

Learn more about surgery for head and neck cancer.

When should I see a doctor?

Contact a doctor as soon as you have concerns about adenoid cystic carcinoma.

You may not experience symptoms of adenoid cystic carcinoma. Contacting your doctor as soon as you notice symptoms will help ensure diagnosis and treatment as early as possible.

View our Cancer Appointment Guide for advice ahead of your appointment.

How do doctors diagnose adenoid cystic carcinoma?

To help reach an accurate diagnosis, your doctor may take a full medical history and perform a physical examination. They will then order tests to confirm the cause of your symptoms.

Tests your doctor arranges may depend on which symptoms you experience. Possible tests include:

  • biopsy to remove a small amount of the affected tissue for examination
  • fine-needle biopsy to remove a small sample of tissue from salivary glands
  • imaging tests, such as an MRI or CT scan to assess the size and location of the tumor
  • lung function tests in suspected cases of tracheal adenoid cystic carcinoma
  • direct laryngoscopy to examine the larynx
  • barium X-ray to examine the esophagus
  • mammogram to examine the breasts
  • cervical smear test in suspected cases of cervical adenoid cystic carcinoma

Your doctor can explain which tests they order in more detail.

How common is adenoid cystic carcinoma?

An adenoid cystic carcinoma is a rare form of adenocarcinoma. It affects around 200,000 people worldwide.

Around 4 in 1 million people annually receive an adenoid cystic carcinoma diagnosis.

Adenoid cystic carcinoma accounts for around 1% of cancerous tumors of the head and neck. It accounts for around 10% of all salivary gland tumors.

Adenoid cystic carcinoma in the breast is extremely rare. It accounts for less than 0.1% of all breast cancers.

What are the complications of adenoid cystic carcinoma?

Adenoid cystic carcinoma typically grows slowly and spreads less frequently than other carcinomas.

However, one possible complication of adenoid cystic carcinomas is recurrence. Following surgical removal, there is a high chance that the tumor will return.

Additionally, metastasis occurs in more than 50% of cases, meaning the cancer will spread to another part of the body. Common areas where the cancer may spread include:

  • lungs
  • bone
  • liver
  • skin
  • breast

Risk factors for recurrence or distant metastasis include a tumor larger than 3 centimeters and the involvement of regional lymph nodes.

Contact your doctor if you have concerns about the complications of adenoid cystic carcinoma.

Learn about bone metastasis.

Can I prevent adenoid cystic carcinoma?

Researchers do not know the cause of adenoid cystic carcinoma. As such, it may not be possible to prevent the condition.

Contact your doctor as soon as you have concerns about adenoid cystic carcinoma to help prevent the tumor from growing or spreading. Your doctor can perform tests and advise on recommended treatments to reduce this risk.

Summary

Adenoid cystic carcinoma is a rare type of adenocarcinoma that typically begins in the skin. It is usually slow to grow and may not cause any symptoms.

Adenoid cystic carcinoma most frequently affects the salivary glands. Symptoms include facial numbness and weakness, swallowing difficulties, and a painless lump in the face, neck, or mouth.

Contact your doctor as soon as you experience symptoms of adenoid cystic carcinoma. They can carry out tests to reach an accurate diagnosis and recommend the best treatments for you.

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Medical Reviewer: Faith Selchick, DNP, AOCNP
Last Review Date: 2022 Sep 15
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THIS TOOL DOES NOT PROVIDE MEDICAL ADVICE. It is intended for informational purposes only. It is not a substitute for professional medical advice, diagnosis or treatment. Never ignore professional medical advice in seeking treatment because of something you have read on the site. If you think you may have a medical emergency, immediately call your doctor or dial 911.
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