Adrenocortical carcinoma is a rare and aggressive carcinoma (cancer arising from cells in the skin or the tissues lining organs) that is classified as a neuroendocrine tumour. These tumours develop in the adrenal glands, which sit on top of the kidneys. Adrenal glands are responsible for producing hormones that help regulate important bodily functions such as metabolism, immune system, and blood pressure.
Neuroendocrine cancers are a complex group of tumours that develop in the neuroendocrine system, which is responsible for regulating important bodily functions such as heart rate, blood pressure and metabolism. They most commonly develop in the gastro-intestinal tract, pancreas, and the lungs; however, they can develop anywhere in the body. These tumours develop from neuroendocrine cells, which are responsible for receiving signals from the nervous system and producing hormones and peptides (small proteins) in response.
Adrenocortical carcinomas can present as either functional or non-functional tumours. Functional tumours are those that secrete extra hormones, while non-functional tumours do not secrete extra hormones.
This type of cancer has a prevalence in females and is most commonly diagnosed in children under 10, or adults between the ages of 40-50. However, this disease can develop at any age in any sex.
Treatment
If an adrenocortical carcinoma is detected, it will be staged and graded based on size, metastasis (whether the cancer has spread to other parts of the body) and how the cancer cells look under the microscope. Staging and grading helps your doctors determine the best treatment for you.
Cancers can be staged using the TNM staging system:
- T (tumour) indicates the size and depth of the tumour.
- N (nodes) indicates whether the cancer has spread to nearby lymph nodes.
- M (metastasis) indicates whether the cancer has spread to other parts of the body.
This system can also be used in combination with a numerical value, from stage 0-IV:
- Stage 0: this stage describes cancer cells in the place of origin (or ‘in situ’) that have not spread to nearby tissue.
- Stage I: cancer cells have begun to spread to nearby tissue. It is not deeply embedded into nearby tissue and had not spread to lymph nodes. This stage is also known as early-stage cancer.
- Stage II: cancer cells have grown deeper into nearby tissue. Lymph nodes may or may not be affected. This is also known as localised cancer.
- Stage III: the cancer has become larger and has grown deeper into nearby tissue. Lymph nodes are generally affected at this stage. This is also known as localised cancer.
- Stage IV: the cancer has spread to other tissues and organs in the body. This is also known as advanced or metastatic cancer.
Cancers can also be graded based on the rate of growth and how likely they are to spread:
- Grade I: cancer cells present as slightly abnormal and are usually slow growing. This is also known as a low-grade tumour.
- Grade II: cancer cells present as abnormal and grow faster than grade-I tumours. This is also known as an intermediate-grade tumour.
- Grade III: cancer cells present as very abnormal and grow quickly. This is also known as a high-grade tumour.
Once your tumour has been staged and graded, your doctor may recommend genetic testing, which analyses your tumour DNA and can help determine which treatment has the greatest chance of success. They will then discuss the most appropriate treatment option for you.
Treatment is dependent on several factors, including location, stage of disease and overall health. There is no one treatment option for this type of cancer, due to the rarity of the disease.
Treatment options for adrenocortical carcinomas may include:
- Surgery, potentially including:
- Adrenalectomy (removal of affected adrenal gland).
- Lymphadenectomy (removal of affected lymph nodes).
- Radiation therapy.
- Chemotherapy.
- Immunotherapy.
- Targeted therapy.
- Clinical trials.
- Palliative care.
For more information on treatment options, please refer to the Rare Cancers Australia Treatment Options page.
Risk factors
The risk factors for adrenocortical carcinoma vary between children and adults.
The risk factors for adults include:
- Li-Fraumeni syndrome (inherited condition that increases the risk of developing cancer).
- Beckwith-Wiedemann syndrome (inherited growth regulation disorder).
- Carney complex (inherited condition associated with skin pigmentation abnormalities and increased risk of developing cancer).
The risk factors for children include:
- Having a genetic mutation of the gene TP53.
- Li-Fraumeni syndrome.
- Beckwith-Wiedemann syndrome.
- Hemihyperplasia (inherited condition involving overgrowth of one side of the body).
Not everyone with these risk factors will develop the disease, and some people who have the disease may have none of these risk factors. See your general practitioner (GP) if you are concerned.
Early symptoms
Symptoms of adrenocortical carcinoma will vary between adults and children, as well as between functional and non-functional tumours.
Paediatric symptoms
Most children with adrenocortical carcinoma have functioning tumours. However, children can also develop non-functional tumours. The symptoms of childhood adrenocortical carcinomas are:
- Abdominal or back pain.
- A lump in the abdomen.
- A feeling of fullness in the abdomen.
- High blood pressure.
- Acne.
- Growing of body hair not associated with regular puberty.
- Deepening of the voice not associated with regular puberty.
- Accelerated growth rate.
- Enlarged genitalia (caused by excess androgen hormone).
- Growth of breast tissue in males (caused by excess oestrogen in males).
- Cushing’s syndrome (caused by excess cortisol).
Non-functional adrenocortical carcinoma in adults
Early stages of a non-functional adrenocortical carcinoma may not cause any symptoms in early stages. As the tumour progresses, symptoms may occur, including:
- Abdominal or back pain.
- A lump in the abdomen.
- A feeling of fullness in the abdomen.
Functional adrenocortical carcinoma in adults
The symptoms of functional adrenocortical carcinomas will vary based on the hormone that is being produced in excess.
Excess cortisol
Excess cortisol production may cause following symptoms:
- Unexplained weight gain (specifically in the face, neck, trunk, and limbs).
- Growth of facial, back or arm hair.
- A round, red, full, face.
- Deepening of the voice.
- Muscle weakness.
- High blood pressure and/or blood sugar.
Excess aldosterone (Conn's syndrome)
Excess aldosterone production is commonly referred to as Conn's syndrome, or primary hyperaldosteronism. When excess aldosterone is produced, the adrenal glands have reduced capacity to produce renin, an enzyme that assists in controlling blood pressure and maintaining healthy levels of sodium and potassium in the body.
Conn's syndrome may cause the following symptoms:
- High blood pressure (hypertension) that is difficult to treat.
- Headaches.
- Nausea and/or vomiting.
- Heart paliptations.
- Low potassium levels in the blood.
- Muscle weakness and/or cramping.
- Frequent urination.
- Constantly feeling thirsty.
Excess testosterone
Excess testosterone production in men will usually present as asymptomatic. Excess testosterone production in women may cause the following symptoms:
- Growth of facial, back or arm hair.
- Acne.
- Hair loss/balding.
- Deepening of the voice.
- Absence of menstrual periods.
Excess oestrogen
Symptoms of excess oestrogen production will vary between men and women. In women, the following symptoms may appear:
- Irregular menstrual periods.
- Vaginal bleeding in women who have gone through menopause.
- Unexplained weight gain.
In men, the following symptoms may appear:
- Growth of breast tissue.
- Decreased or absent sex drive.
- Impotence (inability to develop and/or maintain an erection).
Not everyone with the symptoms above will have cancer, but see your GP if you are concerned.
Diagnosis/diagnosing
If your doctor suspects you have an adrenocortical carcinoma, they will order a range of diagnostic tests.
Physical examination
Your doctor will collect your overall medical history, as well as your current symptoms. Following this, they may examine your body to check for any abnormalities.
Endocrine studies & blood tests
Endocrine studies involve blood and/or urine tests and imaging tests (see below) to analyse your hormone levels and detect any abnormalities. Some of these tests may include:
- General blood test to assess overall health.
- Blood chemistry and/or blood hormone studies, which analyse the levels of certain hormones and other substances in the blood.
- 24-hour urine test to assess cortisol and/or 17-ketsteroid levels.
- Dexamethasone suppression test (high or low dose), where high or low doses of dexamethasone (a type of medication) is given to the patient, followed by blood or urine tests being conducted over the following three days to check cortisol levels.
Imaging tests
The doctor will take images of your body using magnetic resonance imaging (MRI), a computed tomography scan (CT scan), x-ray, and/or positron emission tomography (PET scan), depending on where it is suspected the cancer is. The doctor may also look at other parts of the body and look for signs of metastasis. Your doctor may also recommend an adrenal angiography (looks at arteries) or venography (looks at veins), where a contrast dye is injected into adrenal area being observed to check for any abnormalities, such as blockages.
Biopsy
Once the location(s) of the cancer has been identified, the doctor will perform a biopsy to remove a section of tissue using a needle. The tissue sample will then be analysed for cancer cells. This can be done by a fine needle aspiration (FNA) or a core needle biopsy (CNB).
Prognosis (Certain factors affect the prognosis and treatment options)
While it is not possible to predict the exact course of the disease, your doctor may be able to give you a general idea based on the rate and depth of tumour growth, susceptibility to treatment, age, overall fitness, and medical history. Generally, early-stage adrenocortical carcinomas or children with the disease have a better prognosis and survival rates. However, if the cancer is advanced and has spread, the prognosis may not be as good and there may be a higher risk of cancer recurrence. It is very important to discuss your individual circumstances with your doctor to better understand your prognosis.
References
Some references are to overseas websites. There may be references to drugs and clinical trials that are not available here in Australia.