My aunt has had an operation for a cancer of her adrenal cortex. Can you tell me about these tumours?

There are two adrenal glands in the body. They sit just on top of and in front of the two kidneys. The glands are formed of an outer layer, called the cortex and an inner core, called the medulla.

Although they are small, the adrenal glands are very important as they produce a number of vital hormones.

Tumours of the adrenal gland can develop in either the cortex or the medulla. Adrenal cortical tumours may be either benign (non-cancerous) in which case they are called adenomas, or cancerous (malignant) in which case they are called adrenal cortical carcinomas. Tumours of the medulla are called phaeochromocytomas (pronounced fee-oh-cromo-sigh-toma) and are usually benign but may occasionally be malignant.

Adrenal cortical carcinomas are rare. They account for only about 1 in every 2000 cancers and affect about 2 people in every million, each year. They can occur in young children, under the age of five. When they occur in adults they usually affect people between the ages of 40 to 70.

About two thirds of adrenal cortical carcinomas cause symptoms because of excessive production of one or other of the hormones produced by the adrenal cortex. This can lead to one of three sets of symptoms:

• symptoms due to overproduction of glucocorticoid hormones: this leads to weight gain, with the weight being concentrated on the trunk, whilst their is usually some muscle wasting of the limbs making them appear thinner. There is an accumulation of fat at the base of the neck, giving rise to a 'buffalo hump' appearance and there is swelling of the cheeks and face, giving a 'moon face' appearance. There may also be mild diabetes and abnormal hair growth, with an excess of fine hair growing on the face, arms and upper back. (These symptoms sometimes go under the name of Cushing's syndrome, there are a number of causes for Cushing's syndrome other than adrenal cortical cancers).
• symptoms due to overproduction of mineralocorticoids: this leads to an increase in blood pressure, muscle cramps, thirst and excessive passing of urine. (There are many other causes of raised blood pressure which are very much more common than adrenal cortical cancers).
• symptoms due to overproduction of sex hormones: in women this can lead to masculising tendencies with a deepening of the voice, disturbance of periods and development of male pattern baldness, whereas in men loss of libido and impotence may develop and sometimes some swelling and enlargement of the breasts.

If they do not produce excessive amounts of hormone then adrenal cortical cancers tend to be 'silent' tumours only causing symptoms when they reach quite a large size (10 to 12 cm, or 4 to 5 inches, across) when abdominal or mid back pain, weight loss and tiredness develop. Sometimes the tumours are only discovered when they have already spread elsewhere, causing breathlessness (if the tumour has gone to the lungs), loss of appetite and sickness (if the liver is involved) or bone pain (if the skeleton is affected).

Increasingly these days, adrenal cortical carcinomas are being discovered as a chance finding on CT-scans of the abdomen, which have been done for other reasons, at a stage before they have begun to cause any problems.

The treatment for adrenal cortical carcinomas is surgery to remove the affected gland (and sometimes the nearby kidney and some of the surrounding lymph nodes as well).

The most effective drug in the treatment of these cancers is a chemotherapy drug called o.p-DDD or mitotane, which is used for people who have advanced tumours that have spread to other parts of the body. In this situation it may help to keep the cancer in check for a period of time.

Radiotherapy can also be used to help control any areas of tumour that cannot be removed surgically and will also ease any bone pain due to spread of the cancer to the bones.

Unfortunately adrenal cortical cancers tend to behave aggressively and this, coupled with the fact that they are often discovered at a late stage, means that a cure is only possible for a minority of people with these tumours. However, if it has been possible to completely remove the tumour surgically, a cure is more likely.