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.
History of ACC Research at TGen
Prior developing Kirsten's Legacy, early funding for TGen's ACC program came from ACC survivor Troy Richards, who was instrumental in establishing TGen's ACC program. Richards has actively raised dollars for ACC research through the Advancing Treatment for Adrenal Cancer (ATAC) fund. This program helped bring ACC research out of the dark ages and into the 21st century.
In May 2005, Richards met with TGen faculty to discuss establishing an ACC research program. Richards also developed a website and co-founded what is now the largest ACC support group on the Web. He often receives calls from patients throughout the world, whom he encourages to participate in clinical trials.
Kirsten's Legacy and the ATAC Fund Join Forces
In late 2010, Gary and Barbara Pasquinelli of Yuma, Arizona, along with Kirsten's husband, Ed Sandstrom, began working with TGen to establish Kirsten's Legacy in memory of Kirsten Sandstrom, loving daughter, wife and mother of three, who had lost her battle with ACC in March of that year.
Richards, the Pasquinelli family and Ed merged efforts and today pursue a cure for ACC through the Kirsten's Legacy program.
ACC Tissue Bank
Because ACC is so rare, it is often difficult to collect enough tumor samples to analyze, and yet tissue samples are critical to the advancement of ACC research and to the discovery of better treatments for patients.
With early support from the ATAC research fund, TGen established and now houses the largest "frozen" ACC-specific tissue bank in the world. Tissue banks provide researchers with the high-quality samples necessary to advance laboratory and clinical efforts toward defeating ACC.
This valuable resource enables researchers to study ACC patient samples with a view toward developing better drug therapies and more effective diagnostic tests by identifying those genetic changes that cause ACC.
For questions about how you can donate your tumor for research, please contact our Study Coordinator at [email protected]