Living 18 years with ACC
At 36 years old I was told to get my affairs in order by my urologist. Optimistic, yes, but realistic nonetheless. A football sized tumor was found on my left kidney and the diagnosis was stage 4 adrenal cancer. Such is the nature of Adrenocortical Carcinoma (ACC), a rare cancer of the adrenal glands that usually displays no symptoms or pain, and simply displaces organs as it grows inside the body cavity, often going undetected until its late stages, I was 165 pounds at the time.
ACC occurs in as few as 1-in-1.7 million Americans annually. The disease develops in the adrenal cortex, the outside layer of the adrenal gland. There are two adrenal glands, one above each kidney in the back of the upper abdomen. The adrenal glands are part of the endocrine system, which produces hormones that regulate bodily functions. Surgeons successfully removed the tumor along with one of my kidneys and that’s when the hard decisions needed to be made. There was one drug approved for ACC, it’s called Mitotane, a derivative of DDT. It had a 20% response rate at best and made people very sick with no proof that it would work, I did’t take it and I would never recommend it to an ACC patient today. But with luck, it took four and one half years later and the cancer returned, this time on my left lung. Doctors monitored that tumor for a while, and when they saw it growing, surgeons also removed that tumor, and part of his lung, too.
Now that I had my first recurrence and multiple surgeries, it persuaded me to start looking into research, as my oncologist, Dr. Richard Rosenberg, contacted a colleague at the Arizona Cancer Center, Dr. Daniel Von Hoff, TGen’s Physician-In-Chief. A few days later, I received a call from Dr. Von Hoff, and they talked for 90 minutes about setting up an ACC research program at TGen. Two weeks later, in May 2005, I arrived at TGen for a meeting with Von Hoff and Dr. Michael Demeure, a surgeon who had published a scientific paper about ACC. That day, Drs. Von Hoff and Demeure and I began putting together a plan for what would become TGen’s ACC Research Program.
During the planning process, I developed a tumor in my liver. Surgeons removed the tumor and two-thirds of my liver. But this time the tumor underwent genomic profiling, which revealed a genetic marker that his physicians believed showed the ACC could be treated with Velcade, a drug usually used to treat multiple myeloma. This is where the power of profiling and teamwork comes in. I received the targeted chemotherapy for four months. And except for a spot of cancer on my right lung, removed in May 2008, I remained cancer free until early 2014 and once again the cancer popped back up in the liver. Now in 2014 we can do full Genome sequencing at a fraction of the cost a few years earlier. This time TGen sequenced the tumor and found markers that a drug combination of Temozolomide and Capecitabine would target. I did several months of that chemo regimen and in the fall of 2017 remains free of ACC.
I attribute my remarkable health to the scientific research at TGen lead by Dr. Michael Demeure and Dr. Daniel Von Hoff, my oncologist Dr. Richard Rosenberg, my surgeons and gods good grace. It was not looking real good. I can only say there is a good chance that precision medicine had a major role in my fight. You could say I am living proof that it works, and thinking “outside the box” especially with a rare cancer, is credit to my awesome physicians. I tell my story to give HOPE to those that are facing uncertainty and fear.
Troy and Katarina Richards