Adrenocortical carcinoma
What is cancer of the adrenal cortex?
Adrenocortical carcinoma is a disease in which cancer cells are found in the adrenal cortex, which is the outside layer of the adrenal gland. The function of the adrenal glands is to make important hormones that help the body work properly. When cells in the adrenal cortex become cancerous, they may produce too much of one or more hormones or pre-stages of these hormones. If the tumour makes inactive pre-stages of hormones, the tumour is called "non-functioning", and these tumours constitute around half of all adrenocortical carcinomas.
Adrenocortical carcinoma is a rare disease that affects only 1 to 2 persons per one million population. There is a slight female predominance, and the median age at diagnosis is around
40-50 years. As far as we know today these tumours are not hereditary.
What kind of symptoms may adrenocortical carcinoma cause?
How is the diagnosis made and how is the disease treated?
Symptoms
The clinical symptoms of adrenocortical carcinoma depends on which hormone the tumour produces, and the tumours are divided into groups or syndromes:
Cushing syndrome is caused by hyper secretion of the hormone cortisol. It has typical symptomatology, including hypertension, trunk obesity, moon-shaped face, gain of weight,
oligo-amennorrhagia, sexual malfunctioning, infertility, muscle weakness, osteoporosis and hyperglycaemia.
Adrenogenital syndrome is caused by a tumour which produces excessive amounts of androgens or estrogens. Overproduction of androgens in women leads to hirsutism, deep
voice, acne, amenorrhoea and infertility. In men hyperestrongenism cause gynecomastia, decreased sexual drive, impotence and infertility.
Non-functioning tumours produces inactive hormones and therefore these tumours do not present with any hormonal symptoms. However, the patient can have symptoms caused by the tumour mass, such as pain in the abdomen, loss of weight and weakness. It is common that adrenocortical carcinomas convey metastases to the lungs, the skeleton, lymph nodes, or the liver, and in those cases symptoms such as breathlessness and pain may occur.
Diagnosis
If the tumour is surgically removed, the diagnosis is made through analysis of the removed tumour mass. Otherwise the diagnosis of adrenocortical carcinoma involves the taking of a patient´s medical history and physical examination, followed by laboratory tests of blood as well as urine, aimed at proving overproduction of hormones. To localize the tumour a series of imaging techniques may be employed, computed tomography (CT), ultrasongaraphy, magnetic resonance imaging (MRI) and positron emission tomogaraphy (PET).
Treatment
The aims of treatment are to reduce hormone levels, control hormonal symptoms, prevent further tumour growth and possibly also achieve tumour reduction. Whenever possible systematic surgical removal of all resectable tumour is performed. After surgical removal of the tumour medical treatment often is added. This medical treatment may include chemotherapy, mitotane, antimycotic agents or radiotherapy. Chemotherapy is cytotoxic agents that have a checking or stopping effect on the cell division. Chemotherapy have effect on all cells in the body but mostly on those that have a divide rapidly, such as cancer cells.
There are a number of different sorts of chemotherapeutic agents that are used alone or in combinations. Chemotherapy treatment is always given at the hospital. Mitotane is a drug that inhibits the function of the adrenal cortex which leads to a impaired function or death of tumour cells. Mitotane is taken as pills daily. Antimycotic drugs are used in the treatment of fungus infection, but it also have a inhibiting effect on the hormone production of the adrenal cortex. Radiotherapy is mostly used in the treatment of skeletal metastases.
Research and future outlook
The research work at the department is ongoing, to improve existing techniques for diagnosis and treatment as well as developing new techniques and treatment strategies.
It is important to consider the psychosocial situation for these patients. Research aiming at increasing the knowledge about how patients and there relatives perceive their situation, and how disease and treatment affect the quality of life of these patients is in progress.