ACC with Li-Fraumeni Syndrome
My name is Ann Gallon, I’m 52 years old and was working as a Detective Constable at the time of diagnosis. Pre adrenal cancer I ran 6 days a week. Competing in races; 5ks, 10ks, half marathons, and marathons. Compared to now where I am very fatigued, can’t walk far and just have no energy to do anything.
During October 2021 I suffered from irregular bleeding which my daughter advised me to put in an e-consult with my local GP. The GP decided to send me for an ultrasound since I was under the age of 50 and had a history of breast cancer in 2011. During this – a mass was found and further investigations had to be done.
Over the next few weeks and further scans it was found I had a cancerous tumour on my right adrenal gland. I was told it was most likely secondary to the breast cancer as adrenal cancer is so rare. Only one to two people per million per year being diagnosed.
I had to give blood and urine samples and had many more scans. I was told the tumour had to come out and surgery was scheduled for February 2022.
During this time I sadly lost my mum which in itself was very difficult to deal with.
Whilst at work before my operation I received a phone call from my endocrinologist stating it was looking like adrenocortical cancer (ACC) from the results of the tests. However, this would not be confirmed until after the surgery.
I joined ACC Support UK (AMEND) and their private Facebook Group for ACC. Through this Facebook support group I researched and read all the information (there are not many of us). It was a life-saver to have this group of lovely people who understood. I also read so much information on the AMEND / ACC Support UK websites and listened to others’ stories.
To my surprise my surgeon carried out my surgery by keyhole. I had read that all adrenal surgeries should be done open but I trusted my surgeon. Who wouldn’t? I was told surgery was a success and the tumour along with my adrenal gland was removed. I was put on the oral chemotherapy called “mitotane” which shuts down the remaining adrenal gland making me steroid dependant. I have to carry an emergency steroid injection with me at all times as there is a possibility of me having an “adrenal crisis.” I have my cortisol levels checked regularly and also have routine blood tests.
It was confirmed this was a new primary ACC and was not connected to my breast cancer. Because of this, I was then tested for Li-Fraumeni Syndrome, a rare hereditary or genetic disorder that increases the risk of developing certain types of cancer, including breast and adrenal cancer.
Unfortunately, I tested positive for the very rare Li-Fraumeni Syndrome. My daughter and brother were given the option to have the test which they both did, and thankfully both were negative meaning my granddaughter and my brothers family were also free from the disease. This was a major relief to us all. I returned to work and tried to lead a normal life on my medication.
Then, in June 2023 I was booked in for an ultrasound scan (CT scans are no advised in Li-Fraumeni syndrome). I hadn’t had a check-up since an MRI the previous October. It was the day before my daughter, granddaughter, son-in-law and I were to fly to Orlando for a Disney holiday. I asked the person performing the ultrasound not to disclose anything to me if seen as I wanted to go on holiday and forget.
I had a suspicion something wasn’t right as I was losing weight quite quickly. On my return, I was given the news that a shadow had been found on my liver, and I needed further investigations. Many scans later it was discovered I had tumours in my liver, spleen, stomach, and on the adrenal bed. I was devastated. That meant I was a stage 4 cancer patient.
I remember standing in my parents’ kitchen and my dad saying, “I have lost your mam, I can’t lose you too.”
Meetings were had and it was decided surgery was not an option. I also met my oncologist who didn’t fill me with much hope for the future. I had done my research on my Facebook support group and knew what type of IV chemotherapy was needed and I also knew I was entitled to ask for a second opinion on surgery (respectfully).
I was advised to get my affairs in order, and it was suggested my daughter’s wedding be brought forward. We brought it forward to 7th October and had the most wonderful day. I was so proud to give her away. It was the best day ever. She means the world to me. I had received one round (4 days) of chemo two weeks prior to the wedding and all I wished for was to have hair in the photos. I achieved this although it was coming out in clumps on the morning of the big day.
During the gruelling chemotherapy, which was four days every four weeks, I kept being told it didn’t have a good success rate and to prepare myself for the worst. Four days straight was tough.
Friends of Anchor ‘Aberdeen and North Centre for Haematology, Oncology and Radiotherapy, is a charitable organisation who provide funding for in- and outpatient Wellbeing Services, Medical Equipment and Research, going over and above what the NHS alone can provide. Founded in 1997, the charity is focussed on patient needs within the hospital environment, and their Wellbeing team are on hand to offer a time-out service, and complementary treatments as required. I even had reflexology whilst receiving my chemo. Many of FOC are volunteers and they were amazing! They made my days at chemo that little bit easier.
I requested my surgeon get a second opinion which he was more than happy to do, and he approached a surgeon in Sheffield who is an adrenal specialist. Months went by and the chemo was tough, but I held on to hope for surgery. Sheffield responded requesting up-to-date scans which were done and amazingly the chemo was shrinking most of the tumours, although some had slightly grown. I couldn’t believe when I was told they were discussing my case at their local MDT with the chance of operating.
I travelled to Sheffield with my daughter and granddaughter to meet the surgeon and to have a pre-operative assessment, although the final decision had still not been made. The surgeon went through the many risks of surgery, but I was willing to do anything if it gave me a chance to have more time with my family. I was told I would never be cured but I could be given more time; this was enough for me!
I then got the phone call I had been waiting for – they were going ahead with the surgery. In February 2024 I travelled to Sheffield with my family and went through major surgery. The risks were high. Amazingly the surgeon (my hero) removed 8 tumours all with clear margins. I remained in hospital for 2 weeks recovering. I returned home and ended back in hospital with fluid in my lungs. I eventually recovered and went back to taking mitotane and having three-monthly scans.
If I had not been my own advocate and done my research I would have just been left to carry on. It made me think of just how many others this may have happened to, inspiring me to share my story. Be your own voice – do your research. Medical professionals are amazing and do amazing work but sometimes there are other options than those you are being told about. Request an adrenal cancer specialist as this is such a rare cancer.
Unfortunately, my story doesn’t end on a positive. I have only recently found out I have a lesion on my liver. I’m still waiting for the results of my liver MRI and then a plan will be put in place. Nevertheless, I nearly reached the year mark since surgery in Sheffield which I know saved my life.
I’ll keep being my own advocate and doing the research. The group of amazing people on the ACC Support Facebook Group are my go-to and I hope in turn I give advice when needed. If me sharing my story helps just one person, then that makes me happy.
I just take one day at a time. I have an amazing family support network. I’m very lucky with that. I want to see my granddaughter grow up, so I’ll keep up to date with everything related to ACC thanks to the Facebook group and AMEND/ACC Support UK and continue to fight.
