Treatment for adenoid cystic carcinoma (ACC or AdCC), a rare type of cancer that affects glandular tissue, is determined by your medical care team based on several factors. Treatment typically includes surgical removal of the cancerous tissues along with some form of additional therapy, usually radiation therapy.

This article discusses common treatments for ACC as well as important post-treatment therapies and follow-up.

Surgeries and Specialist-Driven Procedures 

Removing the tumor is the best method of treatment, so long as it’s safe to do so and the expected outcome is positive. There are a few ways of performing the removal surgery. In some cases of advanced stage IV (4) cancer, removal by surgery may no longer be advisable. 

Localized ACC Treatment

In cases where the ACC tumor is localized (meaning it hasn’t spread to other body areas), targeted ACC treatment can be expected. ACC is often found in the salivary glands but can be found in other areas in the head and neck and in other parts of the body where there is glandular tissue.

Types of Specialists

In treating ACC, you may work with a variety of specialists including but not limited to surgeons, radiation oncologists, chemotherapy oncologists, dental practitioners, nutritionists, and rehabilitation and restorative specialists.

The goal will be to remove the tumor and a margin, or area of skin, surrounding it. A surgery is said to be successful if the margin is “clean,” meaning a minimum of 2 millimeters (mm) of cancer-free tissue surrounding the tumor.

Removal surgery can also include smaller branches of nerves if the ACC has spread along your nerve lines. However, it’s standard to spare whatever possible in these surgeries. This means major nerves and any surrounding organs will be avoided as much as possible.

Treatment typically looks like:

Resistance

The rareness of this type of cancer and the slowness of its growth means research opportunities for new treatments have been limited. What is known is that this type of cancer can be resistant to radiotherapy and chemotherapy.

  • Complete tumor removal (the treatment of choice)Post-treatment follow-up or follow-up with radiation therapyIf high-risk for tumor recurrence, you may need ongoing follow-up for monitoring of any signs of abnormal cells returning to the treatment area

The post-removal treatment of radiation or chemotherapy is so that the risk of the cancer coming back (tumor recurrence) or spreading (metastasizing) is reduced. This has remained the standard of care for the past three decades.

A study published in 2021 demonstrated complete cancer remission (cancer-free status) in a patient with a treatment method that combines ​​conventional therapy with the stereotactic radiosurgery boost.

Stereotactic radiosurgery boost side effects (which are typically temporary and get better within a few weeks) include:

What Is Stereotactic Radiosurgery Boost?

A stereotactic radiosurgery boost is when radiation therapy is given by way of a beam of radiation that penetrates your skin without making any incisions or cuts, targeting a small area. The procedure is done in a clinical setting and requires someone to take you home afterward.

  • FatigueSkin irritation at the treatment siteHair loss at the treatment siteHeadacheNeurological symptoms, such as seizure, numbness/tingling, or weaknessGastrointestinal symptoms, such as nausea, vomiting, or diarrhea

Metastatic ACC Treatment Options

When cancer has spread to other organs (i.e., metastases are present), the main treatment includes different types of systemic therapies:

  • ChemotherapyTargeted agents (for example, Herceptin if the tumor is Her2 neu positive)Hormonal treatments (for example, androgen receptor inhibitors if the tumor is positive for androgen receptors)

Sometimes loco-regional approaches or site-specific approaches may be used in conjunction with systemic treatments for palliative purposes (to relieve symptoms but without an aim to cure). Examples of loco-regional treatments include classical radiation therapy (radiotherapy or RT, RTx, or XRT) or newer techniques, including proton radiation therapy.

Follow-Up Treatment

After the first treatment for ACC, follow-up visitation is important so the specialist can examine and determine how the healing process is going (and if treatment was fully successful). 

Who Will Need Chemotherapy?

Chemotherapy is a medication given to people in treatment for cancer. It’s used to stop cancer cells from growing, dividing, and making more cells. Chemotherapy is administered through infusion or injection or given as a pill or topical. While it’s useful in treating many types of cancer, it’s not typically used for treating ACC. It hasn’t been shown to be very effective (radiation has shown better results, too).

The purpose of this is to ensure there are no remaining traces of cancerous cells or new abnormal cells starting to appear. 

One review article published in 2017 in the journal Oral Oncology suggests that clinical follow-up should go on for 15 years or more. This is because of the long period of time in which one can experience a disease recurrence or return with this type of cancer.

Summary

Adenoid cystic carcinoma is typically treated by complete surgical tumor removal when possible. This may be followed by radiation therapy. People at high risk of tumor recurrence may need monitoring for years after treatment.

A Word From Verywell

Cancer treatment can be challenging for the whole family. The thought of ACC returning can be particularly worrisome for people who have reached disease-free status. If you or a loved one are struggling to cope with ACC, reach out to your medical care team for support. They can help with specific treatment symptoms and any future concerns.