Adrenocortical carcinoma (ACC) outcomes: What to expect?

I got started on hydrocortisone within a week of starting Mitotane. This is to prevent what happened to you! Did you not get replacement cortisol started when you started Mitotane? That would be a big mistake on the part of your oncologist and endocrinologist. Some people also need testosterone and florinef to replace missing blood pressure hormones made by the adrenal gland and suppressed by Mitotane.

My tumor was 4.2 centimeters at its largest dimension. Since it was under 5 centimeters then it was staged as stage 2. Are you sure about millimeters or do you mean centimeters?

I was scheduled for an incisional hernia repair and removing the adrenal tumor and gland was just a preventative surgery as no one thought it was cancer. I was told 95% sure it was not cancer. It had been present on CT scans for over 4 years and only recently had started growing. So the general surgeon opened my abdominal, then the endocrine surgeon took out the right adrenal gland and then the general surgeon came back and repaired my hernia and closed my abdominal wall. 6 plus hours total. Everything I read said that a biopsy should not be done as this is a way that the tumor cells can be spread. I’m not sure why a urologist would be in the surgery. An Endocrinologist surgeon is more specific to this surgery. A kidney surgeon might help if the tumor has spread to the kidney but not sure if needed. Same with the liver doctor. Not sure why needed unless there is already spread. How do you know that your son has ACC? I had no symptoms and the CT scans can’t tell you if it is cancerous or not. Treatment is surgical removal, radiation therapy for 5 to 6 weeks and then chemotherapy with a drug called Mitotane(Lysodren) which has 85% rate of nausea, vomiting and diarrhea. The treatment dosage is increased as far as the patient can tolerate. And the length of time varies. Years. It is a pill that you take three times a day. I’m on month 4 and struggling with side effects. Have to take anti diarrhea meds sometimes and nausea meds. I’m also developing cataract fairly rapidly according to my eye Dr. some people require replacement of adrenal hormones such as cortisol, testosterone and blood pressure meds to maintain a normal blood pressure. I’m seeing my oncologist every month for lab and getting a CT scan every 3 months and seeing the endocrinologist every three months.
My hospital stay was a week after surgery because I had other things done. I didn’t know I had ACC until the lab report came back three weeks later. If the surgery is done laparoscopic then it shouldn’t be a long hospital stay. I had a month to recover from surgery before radiation started and then the chemo started when radiation was done. I looked up national standards of care for ACC so I was satisfied that my treatment met the USA and European standards of care. ACC is one in 1.7 to 2 million , so really rare and not much current research on it. If your son is employed then get short term disability started when he has surgery. FMLA if he qualifies. I’m able to move to long term disability because of chemotherapy side effects, but some people are able to work and just take off some time for the surgery and radiation appointments. 5 to 6 weeks of Monday to Friday treatments that last about 15 minutes from when you arrive till you are out the door. The actual radiation is about 2 minutes a treatment. If you have more questions please ask, I am happy to share my experiences.

My son was diagnosed with acc in 2016. He had two surgeries. Second surgery was a carotid artery with resection (positive margins) followed by cyber knife radiation. He had a local recurrence followed with gamma knife. He is now facing another recurrence that is affecting his vision. We were told there is no other treatment and to start thinking about palliative care. Was wondering if there is anyone in this group who has been dealing with long term acc.

My son was diagnosed with acc in 2016. He had two surgeries. Second surgery was a carotid artery with resection (positive margins) followed by cyber knife radiation. He had a local recurrence followed with gamma knife. He is now facing another recurrence that is affecting his vision. We were told there is no other treatment and to start thinking about palliative care. Was wondering if there is anyone in this group who has been dealing with long term acc.

Have you consulted with Dr. Jaydira Del Rivero at NIH?
There are only a few true experts in the country who deal with ACC on a regular basis. She and her surgeon Dr. Hernandez have some amazing success stories. He’s often willing to do surgery when other doctors won’t.
Also, I would highly recommend joining one of the Facebook groups. You will get tons of great advice and connect with many others with ACC. There are some new trials and also some people are having luck with different chemos or a keytruda and Lenvima combo. Some of the other experts are Dr. Habra and Campbell in Texas, Dr. Hammer at the Univ of Michigan and there’s Dr. Antonio Fojo at Columbia Presbyterian University in NYC.
I am a 7 year survivor myself. I have had a reoccurrence in my abdomen that I had surgically removed (sadly I had a biopsy before they told me it was ACC) but no other treatments. I never have taken Mitotane.
Highly recommend the Facebook groups as they are a wealth of knowledge! Just look up adrenal cortical carcinoma and you’ll find them.

Thanks for the info about the doctors and the studies. I’m going to look into facebook groups. I’m still at the point where it is emotionally challenging to do too much research. The old research from the 1990s is so depressing. And hard to find anything else. Your information gives me a place to look!

My surgery was in August 2024, and I had 25 days of radiation from Sept to October. Hospitalization was rough; I recovered well from the surgery and had minimal side effects from the radiation. My Chemo is an oral medication that I take daily for possibly 2 years, 5 years or the rest of my life. In the meantime, I retired from work, so I am adjusting to a different retirement scenario than I had planned. The Chemo has been titrated to my tolerance, and some days, the side effects are still overwhelming. GI side effects, fatigue, brain fog. I have to plan my days not to overdo things energy-wise. I am trying to build relationships with other women to meet for coffee or lunch. I have hobbies that I am ramping up. I also have put a lot of time into getting onto long-term disability from my former employer and setting up Medicare Health Insurance.
I have worked a remote job for the last 11 years, so I have no local former coworkers with whom I can stay in touch. My spouse continues to work and will not likely retire for 4-5 years. I have local kids and their spouses and grandchildren. I hope to get a part-time job to bring in some cash and an outlet to use my nursing skills and interact with people. I see a therapist every 2-3 weeks, and emotionally am on a roller coaster. I use music and videos for emotional support. My spouse is supportive, but he can't be my only support. I see my oncologist monthly with labs and the endocrine dept every 3 months. CT scans every 3 months. One of my biggest emotional struggles is how rare this cancer is and how little recent research there is on it. (1990s) The Chemo treatment is from the 1960s. I feel like a unicorn; even my oncologist rarely sees this cancer. The 5-year survival rates are 60%, but reoccurrence rates are high, so close surveillance is needed. Has anyone survived longer? How long have you been on Mitotane? I just feel so isolated.

@annern Don’t see many recent posts. When you have a one-in-a-million medical adventure it can be lonely. Just joined the club and had a large ACC resected and have not yet started mitotane; hope you’re doing well and stay strong. Wondering how many others are in the same boat.