What is ACC?

What is ACC?

Adrenocortical Carcinoma, also know as ACC, is a rare cancer of the adrenal cortex that affects one to two people per million. The adrenal cortex is the outside layer of the adrenal glands, which are located above each kidney in the back of the upper abdomen. When cells in the adrenal cortex become cancerous, they may overproduce hormones, which can cause symptoms such as high blood pressure, weakening of the bones, or diabetes.

The adrenal cortex produces mineralocorticoids, glucocorticoids, and steroids. The medulla produces epinephrine (adrenaline) and norepinephrine. Together, these compounds regulate many bodily functions, such as blood pressure and heart rate.

Signs and Symptoms

Unfortunately, the signs or symptoms of ACC are not easily identifiable or are often masked by more common ailments. This makes it difficult to diagnose ACC in its early stage. The National Cancer Institute provides a comprehensive list of signs and symptoms.

Because other conditions may cause the same symptoms, a doctor should be consulted if any of these problems occur.

Treatment Options

Currently, surgical removal of the tumor is the only hope for curing ACC. However, in 40-70 percent of patients diagnosed with ACC, the cancer has spread from the primary site to other parts of the body, precluding curative surgery.

The only approved drug  for the treatment of patients with ACC is mitotane. Unfortunately, when given alone, mitotane has a response rate of approximately 20 percent and, unfortunately, adding additional chemotherapies doesn't seem to increase that percentage. That is why the work funded through Kisten's Legacy is critical to continuing the research and clinical trials aimed at understanding and curing ACC.

Where Can I Get More Information?

The National Cancer Institute offers the most comprehensive collection of facts and information dealing with ACC.

We encourage you to visit their website to learn about a wide-range of information surrounding ACC, including treatment and clinical trial options.

Clinical Trials

Even though Mitotane is the only approved drug for the treatment of ACC, there are numerous clinical trials underway. If Mitotane and standard chemotherapy haven't worked, many patients can pursue treatment options through clinical trials.

Information and current news about clinical trials and trial-related data provided by the National Cancer Institute can be found at one of the following links.

Our Mission

The mission of Kirsten’s Legacy is to provide valuable resources to patients and their families while working to cure ACC through identification of new therapies and advancing those discoveries, as quickly as possible, to clinical trials.

How to Get Involved

While ACC is one of most rare and lethal of endocrine cancers, it remains one of the least well funded. Please support our efforts to find new therapies as well as new means of preventing and curing this devastating form of cancer.