What is an adrenocortical carcinoma?
Both benign and malignant tumours can occur in the adrenal glands. An adrenocortical carcinoma is a malignant tumour in the adrenal glands. Malignant means that the tumour grows into nearby healthy tissue and disrupts the function of that healthy tissue. In addition, cells from a malignant tumour can move to other parts of the body and continue growing there: they are then said to have metastasized.
What causes an adrenocortical carcinoma?
It is not known what causes adrenocortical carcinomas. Somewhere in the development of the disease, errors occur in the DNA (the genetic material) of a cell, as a result of which it starts to divide in an uncontrolled manner and take on malignant characteristics. An adrenocortical carcinoma can occur in the context of a hereditary syndrome such as Beckwith-Wiedeman or the Li-Fraumeni syndrome.
How frequently do adrenocortical carcinomas occur?
Adrenocortical carcinomas can occur at any age, but most patients are between 40 and 70 years of age when the disease first presents. Approximately 1 in every million people in the Netherlands is affected by the disease.
An adrenocortical carcinoma can be discovered coincidentally during an echo or a scan of the abdomen (CT or MRI), without any other symptoms being noticed.
The normal function of the adrenal glands is to produce the hormones cortisol and aldosterone, and androgens (sex hormones). In approximately 60% of the cases of adrenocortical carcinomas, the disrupted function of the adrenal glands causes the uncontrolled overproduction of one or more of these hormones. A variety of symptoms can result.
An excess of cortisol can give rise to symptoms similar to Cushing’s syndrome such as a typical distribution of fat around the abdomen (central obesity) and the neck (buffalo hump), muscle loss resulting in thin arms and legs and reduced muscle power, a round and swollen face (moon face), raised blood pressure, thin and fragile skin which bruises more easily than normal, pregnancy ‘ stretch marks on the abdomen and buttocks, tiredness, sleep problems, memory and concentration problems and mood swings such as depression, euphoria, or a psychosis and a raised blood sugar level (diabetes).
An excess of aldosterone can give rise to symptoms such as muscle cramps, headaches, palpitations or heart rhythm disorders, raised blood pressure, a frequent need to urinate, and a low potassium level in the blood.
An excess of androgens can give rise to symptoms such as excess hair growth (hirsutism), a deepened voice, menstruation problems and reduced fertility, excess sweating and skin acne.
Because of its size, and possible in-growth and pressure on other tissues, the tumour can also give rise to other symptoms. The type of symptoms that develop, and their severity, depends on the actual location and the size of the tumour. Examples are pain, nausea and vomiting, breathlessness, and poor bowel movements.
The specialist will carry out supplementary tests if a tumour is found in the adrenal glands to check whether it is malignant and to determine whether there is any overproduction of hormones. .
By means of a scan, usually a CT scan, a check will be made of the location, size and possible enlargement of the tumour, and to see if there are any metastases. In more than one-third of cases, metastases have already developed by the time the diagnosis is made. The definite diagnosis of an adrenocortical carcinoma can only be made after the tumour has been examined and tested by a pathologist. The results of tests carried out on all or part of the tumour will be tested against a number of standard criteria.
Recent research has suggested that a certain concentration of hormones and their waste products in the urine can mean that it is highly probable that the person in question has an adrenocortical carcinoma. At the same time, the absence of these substances in the urine makes it very unlikely that an adrenocortical carcinoma is present. Until there is definite proof of their accuracy, urine tests of this kind are not yet carried out as standard; a number of Dutch hospitals do carry them out however as part of their research programme.
Adrenocortical carcinomas are surgically removed whenever possible. If the tumour is producing hormones at an uncontrolled rate, it is sometimes necessary to take medication prior to the operation to reduce the hormone production. Other points that need attention are the blood pressure, the potassium levels and checking – and if necessary correcting – extreme blood sugar levels.
After the operation, the tumour will be extensively tested; a check will also be made that the tumour has been completely removed. Subsequently, a scan will be taken at regular intervals to make sure the disease has not returned and that no metastases have developed. If the tumour was also producing hormones, further blood and/or urine tests will be carried out to check for overproduction of hormones.
For each patient it will be determined if it is necessary to take mitotane as a follow-up precaution. This medicine is specifically aimed at neutralising any residual malignant cells. The dosage of mitotane needs to be carefully calculated so that just the right amount of the medication is present in the body. The mitotane dosage is monitored regularly on the basis of mitotane levels in the blood. Mitotane has a number of side-effects, the most common being nausea. These side-effects can be treated with other medicines. Mitotane can also cause damage to healthy adrenal cells, whereby the function of the adrenal glands is diminished and adrenal insufficiency can occur. In such cases it is necessary to take hormone replacement medication for cortisol and aldosterone (hydrocortisone and fludrocortisone).
If the tumour could not be removed completely post-operative treatment with mitotane is certainly recommended. A course of radiotherapy is another option. If the tumour grows and/or metastases occur, a specialist (oncologist) will be called in to discuss possible chemotherapy treatment.
If the tumour returns after a longer period of time (a year after the operation, for example) or a few metastases are discovered, doctors will consider a second operation. The aim of this operation will be to remove as much of the tumour as possible, or to reduce the symptoms caused by the metastases. The patient will be started on mitotane and an oncologist will be called in to discuss further treatment options.