What Is Adrenal Cancer?
Your body has two adrenal glands, one located above each kidney.Adrenal cancer usually occurs in the outermost layer of the glands, or the adrenal cortex. A cancerous tumor of the adrenal gland is called an adrenal cortical carcinoma. A noncancerous tumor of the adrenal gland is called a benign adenoma.
The adrenals are small glands that sit above each of the kidneys. The kidneys are located deep inside the upper part of the abdomen.
Each adrenal gland has 2 parts. The outer part, the cortex, is where most tumors develop. The cortex makes certain hormones for the body. These hormones all have a similar chemical structure and are called steroids:
- Cortisol causes changes in metabolism to help the body to handle stress.
- Aldosteronehelps the kidneys regulate the amount of salt in the blood and helps regulate blood pressure.
- Adrenal androgenscan be converted to more common forms of the sex hormones estrogen and testosterone in other parts of the body. The amount of these hormones that result from conversion of adrenal androgens is small compared to what is made in other parts of the body. The testicles produce most of the androgens (male hormones) in men. The ovaries produce most of the estrogens (female hormones) in women.
The inner part of the adrenal gland, the medulla, is really an extension of the nervous system. Nervous system hormones such as norepinephrine and epinephrine (also called adrenaline) are made in the medulla. Tumors and cancers that start in the adrenal medulla include pheochromocytomas (which are most often benign) and neuroblastomas.
Tumors and cancers of the adrenal cortex are covered here, but tumors of the adrenal medulla are not. Neuroblastomas are covered separately elsewhere.
Adrenal cortex tumors
The 2 main types of adrenal cortex tumors are:
- Adenomas(benign or non-cancerous tumors)
- Carcinomas (malignant or cancerous tumors)
These types of tumors can sometimes be hard to tell apart when the cells are looked at under the microscope. Sometimes the only way to know for sure that an adrenal tumor is a cancer is when it spreads to lymph nodes or other organs and tissues. Adenomas do not spread outside the adrenal gland.
Adrenal cortex adenomas
Most tumors of the adrenal cortex are benign tumors known as adenomas. These tumors are usually less than 2 inches (5 centimeters) across. They usually occur in only one adrenal gland, but sometimes both.
Most people with adrenal adenomas have no symptoms and don’t know that they have an adrenal tumor. Some of these adenomas are discovered by accident (incidentally) when CT or MRI scans of the abdomen are done because of an unrelated health problem. About 5% of people who have a CT scan of the abdomen are found to have an adrenal tumor that was not suspected. Many of these are nonfunctional, meaning that they don’t make adrenal hormones. Sometimes these tumors are known by the nickname incidentalomas because they aren’t causing problems and were only found by accident.
Some adenomas make too many adrenal steroid hormones. Sometimes the excess hormones can cause the same symptoms as those from adrenal carcinomas (cancers). To learn more, see Signs and Symptoms of Adrenal Cancers. Adenomas are much more likely than carcinomas to make high levels of aldosterone, which can cause high blood pressure.
Adenomas can be cured by removing the adrenal gland that contains the adenoma. Some adrenal adenomas that cause hormone-related symptoms can be treated effectively with drugs that block the production or actions of these hormones. This may be the best treatment choice for patients with other serious medical problems who might not be able to have a major operation.
The treatment of an adenoma depends on the chance that it may be a cancer and whether or not it is raising hormone levels. When an adrenal tumor is found accidentally, tests are often done to see if it is making hormones. If it is, surgery is often recommended. Otherwise, surgery may only be recommended if it is likely to be a cancer. Small tumors are less likely to be cancer, and are often watched but not treated right away. The CT (or MRI) scan can be repeated in 6 to 24 months to see if the tumor has grown. If it has, it may need to be removed. If it hasn’t grown, hormone levels will be watched over the next few years. If the tumor stays small and doesn’t make any hormones, it might not need to be treated at all.
Adrenal cortex cancer
The type of cancer that develops in the cortex of the adrenal gland is called adrenal cortical carcinoma or just adrenal cancer. This rare type of cancer is also known as adrenocortical cancer (or carcinoma).
Adrenal cancer most often is discovered when:
It is found accidentally on an imaging test done to look for something else.
It makes hormones that cause changes such as weight gain and fluid retention, early puberty in children, or excess facial or body hair growth in women.
It starts causing symptoms because it has gotten very large. Large tumors can press on other organs in the abdomen, causing pain or a feeling of fullness. Generally, adrenal cancers are much larger than adrenal adenomas. An adrenal tumor larger than 5 or 6 centimeters (about 2 to 2 1/2 inches) is assumed to be a cancer. In one study, the average size of an adrenal cancer was about 13 cm (5 inches).
Most cancers found in the adrenal gland did not start there and are not adrenal cancers. Instead, they started in other organs or tissues and then spread (metastasize) through the bloodstream to the adrenal glands. For example, lung cancers, melanomas, and breast cancers often spread to the adrenals. When other cancers spread to the adrenals, they are not considered adrenal cancer. They are named and treated based on the place where they started.
We do not know exactly what causes most adrenal cortical tumors. Over the past several years, experts have made great progress in understanding how certain changes in a person’s DNA can cause cells in the adrenal gland to become cancerous. DNA is the chemical in our cells that makes up our genes, which controls nearly everything the cells do. We usually look like our parents because they are the source of our DNA. But DNA affects more than just the way we look. It also determines our risk for developing certain diseases, including some types of cancer.
- Genes that help our cells grow and divide are called oncogenes.
- Genes that slow down cancer cell division or make them die at the right time are called tumor suppressor genes.
Cancers can be caused by DNA mutations (changes) that turn on oncogenes or turn off tumor suppressor genes. Some people with cancer have inherited DNA mutations from a parent, which increase their risk for developing the disease. But most DNA mutations that are seen in cancers happen during life rather than having been inherited. Some of these mutations may result from exposure to things like radiation or cancer-causing chemicals. But most of these mutations seem to happen for no apparent reason, without having an outside cause.
Some of the DNA mutations that cause adrenal tumors in people with genetic syndromes are discussed in Adrenal Cancer Risk Factors. Overall though, these rarely cause adrenal cortical cancer. However, because adrenal cancer is so rare, if you have adrenal cancer, it may be worthwhile to consider genetic testing to find out if you have one of these syndromes. If you do, you (and your family members) might have an increased risk of developing other cancers also.
The Li-Fraumeni syndrome is caused by inherited mutations that inactivate the TP53 tumor suppressor gene. This syndrome causes a small portion of adrenal cancer in adults (about 1 of every 20), but it’s often the cause of adrenal cancer in children. In fact, about 8 of every 10 cases of adrenal cancer in children are caused by Li-Fraumeni syndrome. Many other adrenal cancers have also been found to have TP53 gene changes that were acquired after birth (not inherited).
Symptoms of adrenal cancer are caused by the excess production of hormones. These are typically androgen, estrogen, cortisol, and aldosterone. Symptoms may also arise from large tumors pressing on organs of the body.
Symptoms of excessive androgen or estrogen production are easier to spot in children than adults because physical changes are more active and visible during puberty. Some signs of adrenal cancer in children can be:
- excessive pubic, underarm, and facial hair growth
- an enlarged penis
- an enlarged clitoris
- large breasts in boys
- early puberty in girls
In about half the people with adrenal cancer, symptoms don’t appear until the tumor is large enough to press on other organs. Women with tumors that cause increases in androgen may notice facial hair growth or deepening of the voice. Men with tumors that cause increases in estrogen may notice breast enlargement or breast tenderness. Diagnosing a tumor becomes more difficult for women with excess estrogen and men with excess androgen.
Symptoms of adrenal cancer that produces excess cortisol and aldosterone in adults can include:
- high blood pressure
- high blood sugar
- weight gain
- irregular periods
- easy bruising
- frequent urination
- muscle cramps
At this point, scientists don’t know what causes adrenal cancer. According to the American Cancer Society, about 15 percent of adrenal cancers are caused by a genetic disorder. Certain conditions can put you at an increased risk of developing adrenal cancer.
- Beckwith-Wiedemann syndrome,which is an abnormal growth disorder marked by a large body and organs. Individuals with this syndrome are also at risk for cancer of the kidney and liver.
- Li-Fraumeni syndrome,which is an inherited disorder that causes an increased risk for many types of cancers.
- Familial adenomatous polyposis (FAP),which is an inherited condition characterized by high numbers of polyps in the large intestines that also carries a high risk of colon cancer.
- Multiple endocrine neoplasia type 1 (MEN1),which is an inherited condition that causes many tumors to develop, both benign and malignant, in tissues that produce hormones like the pituitary, parathyroid, and pancreas.
Diagnosing adrenal cancer usually begins with your medical history and a physical exam. Your doctor will also draw blood and collect a urine sample for testing.
Your doctor may order further tests such as:
- an image-guided fine needle biopsy
- an ultrasound
- a CT scan
- a positron emission tomography (PET) scan
- an MRI scan
- an adrenal angiography
Ablation, or the destruction of tumor cells, may be necessary for tumors that are unsafe to remove surgically.
Mitotane (Lysodren) is the most common drug used in the treatment of adrenal cancer. In some cases, it’s given after surgery. It can block excessive hormone production and may help decrease the size of the tumor. You can also discuss clinical trial treatments with your doctor, such as biologic therapy, which uses the immune system to fight cancer cells.
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