Adrenocortical carcinoma: symptoms, diagnosis and treatment in the UK

The adrenal glands are small glands, one above each kidney, that make hormones vital to life. Cancer Research UK explains that the outer layer, the cortex, makes cortisol, aldosterone and sex hormones, while the inner medulla makes adrenaline and noradrenaline. Adrenal cortical cancer (ACC), also known as adrenocortical carcinoma, starts in the cortex.

There are three types of adrenal gland tumour listed by Cancer Research UK: adrenal cortical cancer, phaeochromocytoma, and adenomas, which are benign. Adenomas are often found by chance on scans for other reasons and most do not make hormones. Secondary cancer in the adrenal glands, spread from elsewhere such as the kidney, is more common than a cancer starting there and is treated according to where it began.

ACC is rare, and for that reason treatment is concentrated in specialist teams.

Cancer Research UK says it is not known what causes most ACC. A small number of people are born with gene changes that increase their risk, and it lists Li Fraumeni syndrome, Lynch syndrome, Beckwith-Wiedemann syndrome, familial adenomatous polyposis, multiple endocrine neoplasia type 1 and Carney complex. These inherited changes are rare.

Symptoms depend on whether the tumour makes hormones. Cancer Research UK says you are more likely to have symptoms if your cancer makes hormones, and that the symptoms depend on which hormone is produced.

Cancer Research UK says you usually need several tests if your doctor thinks you have ACC, which may include blood and urine tests, and scans such as a CT scan or MRI scan. The results help your doctor find out more about the cancer, including the stage, which helps plan the best treatment.

Because adenomas are often found by chance and most do not make hormones, working out whether a mass is benign or cancer, and whether it is making hormones, is part of the assessment.

Cancer Research UK says treatment depends on how big the cancer is, whether it has spread and your general health and fitness. Generally, you have an operation to completely remove the cancer if you have an early stage ACC. Surgery gives the best chance of curing ACC, and you usually have an adrenalectomy, where the surgeon removes the whole affected adrenal gland, and possibly nearby lymph nodes or parts of nearby organs.

After surgery you might have the chemotherapy drug mitotane (Lysodren) to lower the chance of the cancer coming back, taken as tablets for at least 2 years, with regular blood tests to check the dose. Hormones produced by the cancer can make blood pressure unstable around surgery, so you usually have hydrocortisone during and after the operation, sometimes for some time afterwards.

If ACC returns or spreads, you usually have chemotherapy, possibly with mitotane and combinations such as doxorubicin, etoposide and cisplatin, and your doctor might suggest debulking surgery. Local treatments such as radiotherapy, chemoembolisation, radiofrequency ablation, microwave ablation or cryotherapy may be used depending on where it has spread. Cancer Research UK says these treatments will not cure advanced ACC but can help control growth and relieve symptoms.

Cancer Research UK says coping with a diagnosis of a rare cancer can be especially difficult and that being well informed can make decisions and coping easier. Talking to other people who have the same thing can also help, and its Cancer Chat forum and nurse helpline are available.

Cancer Research UK says your doctor checks your hormone levels regularly after surgery to make sure you have all the hormones you need, and that mitotane is monitored with regular blood tests.

On the NHS, suspected adrenal tumours are referred to specialist endocrinology and surgical teams and discussed at an MDT. Cancer Research UK says you might go to a different hospital if there is no specialist MDT locally.

Private care is possible for consultations and scans, but because ACC is rare, the key factor is access to an experienced multidisciplinary adrenal team. Ask whichever provider you use how many people with ACC the team treats and whether your case will be discussed at a specialist MDT.