Adrenal Cancer

Definition

Definition of Adrenal Cancer
The adrenals are small glands that sit above each of the kidneys. The kidneys are found deep inside the upper part of the abdomen.

The adrenal gland is made up of two parts:

1. The cortex (the outer part) is where adrenal cortical tumors start. The job of the cortex is to make certain hormones, called steroids, for the body.
2. The medulla (the inner portion of the adrenal gland) is really a part of the nervous system. Nervous system hormones are made in the medulla.

Symptoms

Symptoms of Adrenal Cancer
In children, symptoms are most often caused by male-type hormones (androgens) that the tumor makes. These symptoms include:

1. Excess growth of facial, pubic, and underarm hair
2. Enlargement of the penis (boys)
3. Enlargement of the clitoris (girls)
4. Women with tumors that make androgens may notice the growth of excess facial and body hair, irregular periods, and deepening of their voice

Causes

Causes of Adrenal Cancer
Risk factors for Adrenal Cancer include:

1. A family history: Most adrenal cancers are not linked to heredity. But a rare condition called Li-Fraumeni syndrome causes a higher risk of several types of cancer, including adrenal cancer
2. Age: Most types of cancer are found more often in older people. But adrenal cancer is found most often among adults around ages 40 to 50 and among children

Diagnosis

Diagnosis of Adrenal Cancer
Functioning adrenal cortical cancers and adenomas are frequently diagnosed because of the symptoms caused by steroid hormones. Patients with Cushing's syndrome need to be evaluated to see if the syndrome is caused by a problem in the adrenal glands, the pituitary gland, or another tumor somewhere else in the body. The first step is measuring the amount of cortisol in the urine (called a 24-hour urinary free cortisol test). This test is sometimes performed while giving the patient an extra dose of steroids to see how the body responds. After this is done, most patients undergo a dexamethasone suppression test where patients are given a high dose of the steroid dexamethasone. In normal patients and in patients with Cushing's syndrome due to a problem in the pituitary gland, a high dose of dexamethasone will cause the levels of cortisol in the blood and urine to decrease. In patients with adrenal tumors or another tumor in the body that produces ACTH, cortisol levels remain high even after a patient receives a high dose of dexamethasone.

In patients with excess levels of aldosterone, patients should be tested for blood levels of the chemical renin. In cases of hyperaldosteronism due to a tumor in the adrenal gland, renin levels will be low. In patients who have elevated aldosterone levels due to a problem with the blood vessels of the kidney (a condition called renal artery stenosis), renin levels in the blood are high.

In addition to tests for increased steroid production, radiographic imaging is an important part of the diagnosis of adrenal tumors. Computed Tomography (CT or CAT) scans are commonly used. CT scans use x-rays to form a three-dimensional picture of the inside of the body. If the adrenal tumor is larger than 6 centimeters (cm) on CT scan, it is much more likely to be an adrenal cancer than an adrenal adenoma. In most cases, CT scans can also differentiate between a normal adrenal gland and adrenal hyperplasia.

Ultrasound is sometimes used in the diagnosis of adrenal tumors. Ultrasounds use sound waves to form a picture of the inside of the body. At times, it can be difficult to tell if an adrenal tumor is an adenoma or a cancer. For tumors that are larger than 3 cm, ultrasound is a good method of telling the difference between the two.

Another type of imaging that is used when it is unclear if an adrenal tumor is an adenoma or cancer is Magnetic Resonance Imaging (MRI). MRI uses magnets to produce a very sharp picture of the inside of the body. Certain types of changes on MRI are more commonly seen in adrenal cancers than adenomas and can be used to tell the two apart.

Positron Emission Tomography (PET) scans use radioactively labeled sugar to find rapidly growing cells within the body. When cells are dividing quickly, they require a lot of energy, and the main source of energy in the body is sugar. Areas of actively dividing tissue will require more sugar than slowly dividing tissue. Because cancer cells are rapidly dividing and growing, they take up more the radioactively sugar than the surrounding tissue and this can be detected by the PET scanner. PET scans have been very useful in detect a number of different types of cancers. Its use in adrenal cancers is still being studied.

Ultimately, the only way to tell for sure if an adrenal tumor is an adrenal adenoma or cancer, part of the tumor must be examined underneath a microscope. In most cases of suspected cancer, this is done by obtaining a biopsy of the tumor. A small piece of the tumor is taken, usually through a needle, and examined underneath a microscope. In the case of adrenal tumors, this procedure is usually performed while the patient is undergoing a CT scan, so that the radiologist can see where the needle is going in the body. In some cases, this can also be done using an ultrasound to guide the biopsy.

Treatment

Treatment of Adrenal Cancer
After the cancer is diagnosed and staged, your doctor will talk to you about your treatment choices. The main methods of treatment for adrenal cancer are surgery, radiation therapy, chemotherapy, and the use of other drugs.

Surgery:

1. To remove most adrenal cortical tumors, the surgeon will make an incision through the front of the abdomen
2. If the cancer has grown into the kidney, it will be removed as well
3. If it has grown into the muscle and fat around the gland, these tissues will also be removed
4. If the liver is involved, part of that organ will be removed
5. And if the cancer has grown into the large vein that carries blood from the lower part of the body to the heart, an extensive operation will need to be done

Radiation Therapy: This treatment uses high-energy radiation to kill cancer cells. But radiation therapy is not often used for adrenal cancer because the x-rays do not easily kill this type of cancer.
Chemotherapy: Chemotherapy refers to the use of drugs to kill cancer cells. Usually the drugs are given into a vein or by mouth. Once the drugs enter the bloodstream, they reach throughout the body. Chemotherapy is not very good treatment for adrenal cancer. It is only used to treat adrenal cancer that has become widespread.

Prognosis

Prognosis of Adrenal Cancer
In most cases, when other cancers spread to the adrenal glands, they are treated with chemotherapy that is known to be effective against the original cancer type. In some cases, if the adrenal gland is the only site in the body where the cancer appears to have spread, surgical resection of metastatic cancer and the adrenal gland can be performed, followed by treatment to the primary site where the cancer started. This has been shown to be curative in a small number of patients, particularly in the case of lung cancer.

Prevention

Prevention of Adrenal Cancer
Given that the causes of adrenal cancers are unclear, there are no known interventions that can reduce the risk of developing them.