Adrenocortical carcinomacancer arising from tissues that line organs is a rare and aggressive type of cancera disease where abnormal cells split without control and spread to other nearby body tissue and/or organs that is classified as a neuroendocrine tumoura tissue mass that forms from groups of unhealthy cells. 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 bloodthe red bodily fluid that transports oxygen and other nutrients around the body 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, pancreasa long, flat organ that sits between the stomach and the spine that plays a key role in digestion and blood sugar regulation, and the lungs; however, they can develop anywhere in the body. These tumours develop from neuroendocrine cellsthe basic structural and functional unit of all living things, 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 tumourstumours that produce and secrete hormones or non-functional tumourstumours that do not produce and secrete 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, metastasiswhen the cancer has spread to other parts of the body, also known as mets, and how the cancer cells look under the microscope. Stagingthe process of determining how big the cancer is, where it started and if it has spread to other areas 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 (nodea small lump or mass of tissue in your body) indicates whether the cancer has spread to nearby lymph nodessmall bean-shaped structures that filters harmful substances from lymph fluid.
- 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 tissuea group of cells that work together to perform a function.
- 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 localisedaffecting only one area of body 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 advancedat a late stage, far along or metastatic cancer.
Cancers can also be graded based on the rate of growth and how likely they are to spread:
- Gradea description of how abnormal cancer cells and tissue look under a microscope when compared to healthy cells 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 testinga procedure that analyses DNA to identify changes in genes, chromosomes and proteins, which can be used to analyse tumour DNA to help determine which treatment has the greatest chance of success, 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:
- Surgerytreatment involving removal of cancerous tissue and/or tumours and a margin of healthy tissue around it to reduce recurrence, potentially including:
- Adrenalectomyremoval of one or both adrenal glands.
- Lymphadenectomysurgical removal of lymph node(s).
- Radiation therapya treatment that uses controlled doses of radiation to damage or kill cancer cells.
- Chemotherapya cancer treatment that uses drugs to kill or slow the growth of cancer cells, while minimising damage to healthy cells.
- Immunotherapya treatment that uses a person's immune system to fight cancer.
- Targeted therapymedication that targets specific molecular features of cancer cells.
- Clinical trialsresearch studies performed to test new treatments, tests or procedures and evaluate their effectiveness on various diseases.
- Palliative carea variety of practices and exercises used to provide pain relief and improve quality of life without curing the disease.
Risk factors
The riskthe possibility that something bad will happen 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.
Some of the information regarding risk factors was obtained from the Adrenocortical Carcinoma Treatment (PDQ) and Childhood Adrenocortical Carcinoma Treatment (PDQ) pages published by the National Cancer Institute.
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 abdomenstomach, stomach area, belly.
- A feeling of fullness in the abdomen.
- Hypertensionhigh 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 hormonea chemical substance produced by glands in the endocrine system that regulates various functions in the body).
- 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.
- Hypertension.
- Hyperglycaemiahigh 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:
- Hypertension that is difficult to treat.
- Headaches.
- Nauseato feel sick or likely to vomit and/or vomiting.
- Heart palpitations.
- Hypokalaemialow levels of potassium in the blood.
- Muscle weakness and/or cramping.
- Polyuriafrequent 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.
- Amenorrhoeaabscense 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.
Some of the information regarding symptoms was obtained from the Adrenocortical Carcinoma Treatment (PDQ) and Childhood Adrenocortical Carcinoma Treatment (PDQ) pages published by the National Cancer Institute.
Diagnosis
If your doctor suspects you have an adrenocortical carcinoma, they may order the following tests to confirm the diagnosisthe process of identifying a disease based on signs and symptoms, patient history and medical test results and refer you to a specialist for treatment:
- Physical examinationan examination of your current symptoms, affected area(s) and overall medical history.
- Endocrine studiesstudies that involve blood, urine and/or imaging tests to analyse hormone levels.
- Imagingtests that create detailed images of areas inside the body tests, potentially including:
- X-raya type of medical imaging that uses x-ray beams to create detailed images of the body .
- MRI (magnetic resonance imaging)a type of medical imaging that uses radiowaves, a strong magnet and computer technology to create detailed images of the body.
- CT (computed tomography) scana type of medical imaging that uses x-rays and computer technology to create detailed images of the body.
- PET (positron emission tomography) scana type of medical imaging that uses radioactive tracers to create detailed images of the body.
- Ultrasounda type of medical imaging that uses soundwaves to create detailed images of the body .
- MIBG scana type of medical imaging that uses radioactive tracers to detect overactive parathyroid glands or neuroendocrine tumours; also known as a MIBI or sestamibi scan.
- Adrenal angiographya type of medical imaging that uses x-rays to create detailed images of arteries and assess blood flow and/or venographya type of medical imaging that uses x-rays to create detailed images of veins and assess blood flow.
- Biopsyremoval of a section of tissue to analyse for cancer cells.
