Home » Rare diseases » Effective support and information for ACC is essential

Catherine Bouvier (left) is CEO & Co-Founder of the Neuroendocrine Cancer UK, and President of the International Neuroendocrine Cancer Alliance. Mrs Jo Grey (right) is CEO of AMEND (Association for Multiple Endocrine Neoplasia Disorders)

Adrenocortical Cancer (ACC) is an ultra-rare cancer of the adrenal gland cortex affecting fewer than two people per million, each year. A new test was recently developed that should improve diagnosis.

What is ACC?

ACC is an extremely rare and often aggressive cancer in the outer layer (cortex) of the adrenal gland, affecting about one-to-two people per million, per year. ACC in adults tends to occur in people in their 50s and 60s and is more common in women than in men. Most ACC’s are sporadic (meaning that they do not run in families), but they may sometimes be part of a congenital (present at birth) and/or familial (passed down in families) condition. The cancers can be insidiously silent (non-functioning) or functioning (producing hormones), with symptoms of the latter being wide-ranging; from excess facial or body hair in women or soreness and increase in the size of the breasts in men through to pain, swelling or weight loss. Prognosis is often poor (five-year survival between 35-50%), due to late diagnosis, recurrence, and a lack of effective treatments or clinical trials.

Two recent articles in The Guardian, by Elliott Dallen, highlight the potential aggressiveness and intractability to treatment of ACC. Elliott was diagnosed with ACC aged 29, and died aged 31 on 7th September 2020; the day his second, poignant article was published: “At 31, I have just weeks to live. Here’s what I want to pass on”.

Like many cancers and rare diseases, early diagnosis is key to improving patient outcomes and, whilst this is historically poor in ACC, improvements in diagnostic testing have recently been made which should see this improve and save lives.

How ACC Support got started

After being approached at a medical conference by a surgeon whose sense of desperation at the lack of support for the ultra-rare adrenal cancer community was palpable, the Association for Multiple Endocrine Neoplasia Disorders (AMEND) agreed a collaboration with Neuroendocrine Cancer UK (NCUK) in order to share resources to benefit those with ACC. The result – ACC Support UK – provides them with free information, resources and support services (including Counselling Service and specialist nurse helpline). AMEND also funds ACC research. Recently, three patient experience videos were produced to raise awareness of ACC; in which, some of those affected share their stories of diagnosis, treatment, and life with ACC.

The videos include Emma, who struggled with horrendous symptoms for three years (even undergoing an unnecessary hysterectomy) before her ACC diagnosis in 2013.

I started getting hair on my chest, on my back, the hair on my head started getting very thin. I went to the doctors probably about 15 times. They took bloods but thought it was hormonal issues. It got so bad that I started wearing wigs.


After surgery to remove her adrenal gland and tumour, and a section of liver containing metastatic disease, Emma was put on a combination of medical therapies to manage the residual cancer and maintain her adrenal function. Although her disease has thankfully not yet recurred, the thought of this happening is still something that, understandably, regularly concerns Emma.

The future for ACC patients

Progress is happening thanks international expert clinicians and researchers. For example, a collaborative group of researchers (European Network for the Study of Adrenal Tumours / ENS@T), recently showed that by utilising a specific triad of screening modalities, including a new urine metabolomics test, ACC can be accurately identified from other, non-malignant adrenal nodules. In addition, a new blood test to measure circulating cell-free DNA is currently being investigated to help diagnose and detect recurrence of ACCs earlier than is presently possible.

Furthermore, care for people with ACC in the UK is due to be centralised soon as part of the National Specialised Commissioning initiative, meaning that they will be able to receive treatment and care only from expert centres. All of these dedicated efforts promise earlier diagnosis, improved prognosis, and hope for all those affected by ACC in the future.

New diagnostic urine test: Lancet Article (2020)
Research into Circulating Cell Free DNA: Project funded by AMEND

This article has been commissioned and fully funded by HRA Rare Diseases. HRA RD have reviewed the content for medical accuracy only. The narrative and views expressed are the authors own. Any links visited from this site are not the responsibility of HRA Pharma Rare Diseases. UK/ENDO/0058 September 2020

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