Symptoms of Adenoid Cystic Carcinoma

Specific symptoms of adenoid cystic carcinoma (ACC or AdCC) depend upon the glands affected by this rare form of cancer. In most cases, ACC begins in the head and neck and invades salivary glands (the glands in your mouth that make and release saliva).

Lesions or painless masses (cell growths) in the salivary glands are signs of ACC. This article describes the signs and symptoms of adenoid cystic carcinoma and associated complications.

Frequent Symptoms

Frequent signs or symptoms usually involve painless tumor development in the salivary glands. You may not notice any signs of ACC for years. Your doctor or dentist may be the one to notice any changes first. 

Tumors grow slowly. Common tumor locations include:

• Parotid glands (inside each cheek)
• Submandibular glands (in the mouth floor)
• Sublingual glands (below the tongue)

ACC can also develop in the minor or microscopic salivary glands. These include glands within the following areas:

• Mouth (roof or floor under the tongue)
• Tongue and lip lining
• Inside the cheeks
• Nose and sinuses
• Larynx, or voice box

When symptoms do occur, you can expect them to be linked directly to the cancerous tumor. For example, pain and nerve dysfunction follow when the tumor grows. Eventually a tumor may make its way into your nerves, causing severe symptoms.

Examples of salivary gland ACC symptoms include:

• Weakness or numbness in the face, neck, jaw, or mouth
• Persistent dull pain in the face, neck, jaw, or mouth
• Difficulty opening your mouth fully or moving your facial muscles (facial paralysis or freezing) 
• Trouble swallowing
• Bleeding from the mouth
• Voice hoarseness 
• Abnormal area on the mouth lining

Site-Specific Symptoms

The location of the tumor can result in these site-specific symptoms:

• Salivary gland: Numbing of lower lip or other facial areas; mouth or face masses
• Tear gland: Tear gland swelling or vision changes
• Scalp or ear canal: Pain and discharge of pus or blood
• Skin: Skin plaques like those found in cases of psoriasis (a skin condition with itchy red patches sometimes with silvery scales) and heightened pain sensitivity or pain sensations from things not normally associated with pain (like clothing tags or hugs)
• Trachea (windpipe) or throat: Breathing difficulties, hoarseness, high-pitched respiratory sound upon the intake of air (stridor), feeling unwell, weight loss, pain, recurrent inflammation of the lungs (pneumonitis), coughing up blood
• Larynx (voice box): Difficulty breathing upon exertion, shortness of breath, and eventual windpipe (airway) obstruction
• Esophagus (food tube): Difficulties with swallowing soft foods as well as liquids
• Breast: Considered less severe than other breast cancers but occurs in only one breast; also more likely than other ACCs to spread and to return years after tumor removal
• Cervix: Vaginal bleeding or bloody discharge associated with relatively large cervical masses (cancer is aggressive)
• Prostate: Poor urine flow, increased need to urinate or feeling the need to urinate, enlarged prostate gland that makes it hard to start peeing
  

Complications

Other symptoms associated with advanced stages of ACC in the salivary glands include tumor growth and spreading to other body areas. Side effects from cancer treatments can also be considered complications.

The American Society of Clinical Oncology (ASCO) says side effects will depend on many factors, including:

• Cancer stage
• Length of treatment
• Treatment dose
• General health

Coping with these complications is a part of the treatment plan that your doctor will prepare for you.

Tumor Spread

Tumors can multiply and spread from their point of origin to other areas of the body. This is known as distant metastasis. Oral cancer is usually discovered in a distant location after this spread from its original site. In later stages, patients with oral cancer may then be diagnosed with cancer in the lymph nodes, brain, or lung.

ACC in the respiratory tract can move or metastasize to the lymph nodes. ACC may spread along nerves to bones, particularly the spine (vertebrae). It’s most likely to spread from the origin points to the lungs, with the liver being the second most common site of spread.

Recurring Tumor

ACC is also a kind of cancer that just keeps coming back (recurring). This happens in cases in which small areas of cancer cells go undetected. Over time, these cells keep multiplying (getting larger in number). Eventually, they may cause symptoms to start up again.

One analysis of 90 patients with salivary gland cancer (64% of whom had adenoid cystic carcinomas) suggests distant metastases may occur in 24% of cases at the five-year follow-up and 28% at the 10-year follow-up.

This is why it’s important to attend any follow-up care appointments with a dentist who specializes in oral cancer (called an oncologic dentist). This doctor will monitor for any signs of a tumor returning after radiation. Tumor recurrence can happen years after successful treatment.

Disease return is graded by the following items on the TNM scale :

• Tumor stage (0-5): Indicates whether the tumor has spread and the size of the tumor (T) 
• Indicates if the cancer has spread to nodes (N) 
• Indicates if the cancer has metastasized to distant lymph nodes or organs (metastasized) (M)

Late-Stage Discovery

A severe complication is the relatively high death rate associated with oral cancers (part of head and neck cancers). The high death rate isn’t because the cancer is hard to discover or diagnose. It's due to the cancer being commonly discovered late in its development (after it's had more opportunity to spread). 

In an analysis published in 2013 on follow-up tumor return rates, overall survival rates were 76% and 63% at five and 10 years, respectively.

According to the Oral Cancer Foundation that statistic was still accurate in 2019 because there is no comprehensive program in the United States to do early screening. Without early screening, later-stage discovery is common and complications often increase.

Side Effects from Cancer Treatments

It's typical to have surgery for tumor removal, followed by radiation or chemotherapy. Any treatment method you and your doctor discuss has benefits and risks. These will also be influenced by factors like your general health and tumor size.

Chemotherapy side effects may include:

• Nausea
• Mouth sores
• Hair loss

Radiation side effects when treating head and neck cancers include:

• Fatigue
• Hair loss
• Nausea 
• Mouth sores and taste bud changes
• Skin changes
• Throat changes (trouble swallowing)
• Thyroid gland changes (part of metabolism and body temperature regulation among other bodily functions)

When to See a Doctor

You should see a doctor if you experience any symptoms of salivary gland ACC or if you see signs like a tumor or lesion in the mouth (especially if symptoms have lasted more than two weeks).

Even if there is no pain in the area, visiting a medical professional can help rule out other causes like infection or get you an early diagnosis, which would help with your overall outcome. 

You should also see a doctor if:

• You are experiencing anxiety about your symptoms and the potential of a cancer diagnosis.
• You are having other symptoms related to the stress of living with ACC.
• You are having new or worsening side effects from treatment.
• You’ve noticed a sudden change to your health.
• You think you feel a tumor recurring or symptoms returning after having been symptom free and tumor free for some time.

Summary

Adenoid cystic carcinoma begins growing in glands that produce saliva, most commonly in the head and neck but possibly in other areas of the body. It often has few symptoms while confined to those glands, although a painless tumor might be felt.

It can spread along nerves and through lymph nodes to other sites. This can result in nerve symptoms such as weakness, numbness, and pain. ACC is often not discovered until after it has spread to distant organs and produced symptoms related to those organs.

A Word From Verywell

If you’ve noticed any changes in your oral health, see your dentist or doctor who can refer you to the right type of specialist. While the signs can be scary, remember this type of cancer is relatively uncommon and there’s likely another (noncancer-related) explanation for your symptoms.

Finding the right diagnosis is worth the effort of getting the best treatment. If you have concerns or are experiencing anxiety about your symptoms, do tell your doctor. A doctor can offer advice and help you find support groups of like-minded people.