Adrenal cancer: symptoms, causes, diagnosis and treatment

There are two adrenal glands, one above each kidney. Cancer Research UK explains that they make hormones that help the body work properly and are vital to life. Each gland has two layers: the outer cortex, which makes hormones such as cortisol, aldosterone and sex hormones, and the inner medulla, which makes adrenaline and noradrenaline.

Cancer Research UK describes three types of adrenal gland tumour: adrenal cortical cancer (ACC), phaeochromocytoma, and adenomas, which are benign. Most phaeochromocytomas are non-cancerous. ACC is very rare.

Secondary cancer in the adrenal glands, which has spread there from elsewhere such as the kidney, is more common than cancer that starts in the adrenal glands. Cancer Research UK says that in that case you have the same treatment as for the original cancer, not adrenal gland cancer treatment.

Cancer Research UK says that you are more likely to have symptoms if the cancer makes hormones, and that the symptoms depend on which hormone the tumour produces. Some tumours do not make hormones and might not cause symptoms until they are advanced.

University Hospitals Birmingham NHS Foundation Trust adds that most people are referred to its adrenal tumour service because of abnormalities found on imaging done for other reasons, and that the majority of these are benign. It asks people who develop signs of excess adrenal hormones to contact their GP or hospital team.

Cancer Research UK says it is not known what causes most ACC. A small number of people are born with inherited gene changes that increase their risk. It lists Li Fraumeni syndrome, Lynch syndrome, Beckwith-Wiedemann syndrome, familial adenomatous polyposis, multiple endocrine neoplasia type 1 (MEN1) and Carney complex.

These inherited gene changes are rare, so most people with adrenal cancer have no family history that explains it.

Cancer Research UK says you usually need several tests if your doctor thinks you have ACC. These 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.

The Birmingham adrenal service notes that most abnormalities in the adrenal gland are discovered during imaging for other reasons, and that in a small percentage they produce excess hormones or show aggressive cancers. This is why a referral to a specialist adrenal service is advised when an adrenal abnormality is found.

Cancer Research UK says a team of doctors and other professionals who specialise in ACC, called a multidisciplinary team (MDT), discuss the best treatment and care for you, and that you might go to a different hospital if there is no specialist MDT locally. Treatment depends on how big the cancer is, whether it has spread, and your general health and fitness.

Surgery gives the best chance of curing ACC. You usually have an operation called an adrenalectomy, in which the surgeon removes the whole of the affected adrenal gland and might also remove nearby lymph nodes or part of nearby organs such as the liver, bowel, pancreas or stomach.

You might have a chemotherapy drug called mitotane after surgery to lower the chance of the cancer coming back. Cancer Research UK says mitotane is taken as tablets 2 or 3 times a day, possibly for at least 2 years, with regular blood tests to check the dose.

Because hormones made by the cancer can make blood pressure unstable during and after surgery, you usually have hydrocortisone during surgery and afterwards, and may need it for some time.

Cancer Research UK says that if ACC comes back or spreads, you usually have chemotherapy. Common regimens include doxorubicin, etoposide and cisplatin, gemcitabine and capecitabine, or streptozocin, and mitotane is also used. Doctors might suggest debulking surgery, which removes as much of the cancer as possible, mainly if the advanced cancer makes a lot of hormones and causes symptoms.

Depending on where the cancer has spread, local treatments such as radiotherapy, chemoembolisation, radiofrequency ablation, microwave ablation or cryotherapy may be used. These treatments will not cure ACC, but can help to control its growth and relieve symptoms.

An adenoma is a benign tumour that can start in the cortex. Cancer Research UK says adenomas are often found by chance when people have scans for other reasons, and doctors sometimes call them adrenal incidentaloma. Most do not make hormones, but some make cortisol or aldosterone.

You might not need treatment for an adenoma. Cancer Research UK says you usually only need it if the tumour is growing or making large amounts of hormones.

Adrenal cancer is rare, so care is usually provided by specialist teams that include endocrinologists, adrenal surgeons, oncologists, radiologists and clinical nurse specialists. The Birmingham service lists these roles as part of its multidisciplinary team.

A GP can refer you to an endocrine or adrenal service if symptoms or scan findings suggest an adrenal problem. Private endocrine consultations can be used for an earlier assessment or second opinion, but treatment decisions for a cancer this rare are best made with a specialist MDT.