Risk Factors

Doctors can't always explain why one person gets cancer and another doesn't. However, certain risk factors may make a person more likely than others to develop adrenocortical carcinoma. A risk factor is something that may increase the chance of getting a disease.

Having a risk factor does not mean a person will get adrenocortical carcinoma. Many people who get adrenocortical carcinoma have none of these risk factors, and many people who have known risk factors never get cancer. A person with one or more of these risk factors should talk with his or her doctor about cancer risk.

Risk factors for adrenocortical carcinoma include having any of the following hereditary diseases:

• Li-Fraumeni syndrome
• Beckwith-Wiedemann syndrome
• Carney complex

Symptoms

Adrenocortical carcinoma may cause these common symptoms:

• Pain in the abdomen or back
• A lump in the abdomen

A tumor of the adrenal cortex may be functioning (makes more hormones than normal) or nonfunctioning (does not make hormones). The hormones made by functioning tumors may cause symptoms such as the following:

• A deepening of the voice
• A lump of fat on the back of the neck
• A round, red, full face
• Acne
• Balding
• Feeling thirsty
• Frequent urination
• Growth of fine hair on the face, upper back, or arms
• Impotence
• Irregular or no menstrual periods in women who have not gone through menopause
• Loss of sex drive
• Menstrual bleeding in women who have gone through menopause
• Muscle weakness or cramps
• Swelling of the sex organs or breasts
• Weight gain in the face, neck, or trunk while arms and legs remain thin

Most often, these symptoms are not due to cancer. People with these symptoms should tell their doctor so any problems can be diagnosed and treated as early as possible.

Screening and Diagnosis

Currently, there are no screening methods for adrenocortical cancer.

If symptoms suggest adrenocortical carcinoma, the doctor will try to find out what's causing the problems. The doctor may order one or more of the following tests:

• Physical exam and history: The doctor will check for signs of disease, such as high blood pressure, lumps, or anything else that seems unusual.
• Urine tests: The lab checks urine for levels of hormones made by the adrenal cortex.
• Dexamethasone suppression tests: The doctor gives the patient doses of dexamethasone (a steroid hormone made by the adrenal glands) and collects their blood or urine for three days to look for problems with the adrenal gland.
• Blood tests: The doctor sends blood to the lab to measure hormone levels and to check for certain substances, such as blood sugar, potassium, or sodium released into the blood by organs and tissues in the body. A higher or lower than normal amount of a substance can be a sign of disease.
• CT scan: An x-ray machine linked to a computer takes a series of detailed pictures of the inside of the body. The patient may receive contrast material by mouth or by injection into a blood vessel. The contrast material makes abnormal areas easier to see.
• MRI: A strong magnet linked to a computer is used to make detailed pictures of areas inside the body. Sometimes contrast material is given by injection into a blood vessel. The contrast material makes abnormal areas show up more clearly on the picture. An MRI of the abdomen may be done to diagnose adrenocortical cancer.
• Adrenal angiography: A contrast dye is injected into the adrenal arteries. As the dye moves through the blood vessels, a series of x-rays are taken to see if any arteries are blocked and how blood is flowing near the adrenal gland.
• Adrenal venography: A contrast dye is injected into an adrenal vein. As the contrast dye moves through the vein, a series of x-rays are taken to see if any veins are blocked and how blood is flowing near the adrenal gland. A thin tube may be inserted into the vein to take a blood sample, which is checked for abnormal hormone levels.
• PET scan: The patient receives an injection of a small amount of radioactive sugar. The radioactive sugar gives off signals that the PET scanner picks up. The PET scanner makes pictures of the sugar used by cells in the body. Cancer cells use sugar faster than normal cells, so areas with cancer look brighter on the pictures.

Staging

If adrenocortical carcinoma is diagnosed, the doctor needs to learn the extent (stage) of the disease to help choose the best treatment.

Staging is a careful attempt to find out whether the cancer has spread within the adrenal gland or to other parts of the body.

The doctor may order one or more these staging tests:

• CT scan: An x-ray machine linked to a computer takes a series of detailed pictures of the inside of the body. The patient may receive contrast material by mouth or by injection into a blood vessel. The contrast material makes abnormal areas easier to see.
• MRI: A strong magnet linked to a computer is used to make detailed pictures of areas inside the body. Sometimes contrast material is given by injection into the blood vessel. The contrast material makes abnormal areas show up more clearly on the picture.
• Adrenal angiography: A contrast dye is injected into the adrenal arteries. As the dye moves through the blood vessels, a series of x-rays are taken to see if any arteries are blocked and how blood is flowing near the adrenal gland.
• Adrenal venography: A contrast dye is injected into an adrenal vein. As the contrast dye moves through the vein, a series of x-rays are taken to see if any veins are blocked and how blood is flowing near the adrenal gland. A thin tube may be inserted into the vein to take a blood sample, which is checked for abnormal hormone levels.
• Cavagram: This procedure looks at the inferior vena cava, a large vein that carries blood from the legs and feet and from organs in the abdomen and pelvis and empties into the heart. A contrast dye is injected into a blood vessel. As the contrast dye moves through the blood vessel to the inferior vena cava, a series of x-rays are taken to see if there are any changes to the inferior vena cava and how blood is flowing through the inferior vena cava.
• Ultrasound: The ultrasound device uses sound waves that can't be heard. The sound waves make a pattern of echoes as they bounce off internal organs. The echoes create a picture of the internal organs.
• Adrenalectomy: A surgeon removes the adrenal gland. The doctor in the lab looks at the tissue under a microscope to check for signs of cancer.

Stages of Adrenocortical Carcinoma

Stage I: The tumor is 5 centimeters or smaller and is found only in the adrenal gland.

Stage II: The tumor is larger than 5 centimeters and is found only in the adrenal gland.

Stage III: The cancer may have spread to fat or lymph nodes near the adrenal gland.

Stage IV: The cancer has spread to lymph nodes and to the fat or organs near the adrenal gland. The cancer may have spread to other parts of the body.

Treatment

At Huntsman Cancer Institute, adrenocortical carcinoma is treated by a team of specialists, including endocrinologists (doctors who specialize in diagnosing and treating hormone disorders), urologists (doctors who specialize in diseases of the kidneys and urinary system), surgeons, medical oncologists (doctors who treat cancer with medicine), radiation oncologists (doctors who treat cancer with radiation), nurses, dietitians, and social workers.

Common treatment options for adrenocortical caner include the following:

• Surgery
• Chemotherapy
• Radiation therapy
• Clinical trials

A patient may have a combination of treatments. The treatment that's right each patient depends on many factors:

• Whether the tumor can be completely removed in surgery
• Whether the tumor has spread to other parts of the body
• The patient's age and general health

The health care team can describe all of the treatment choices, the expected results of each, and the possible side effects. Because cancer therapy often damages healthy cells and tissues, side effects are common. Side effects may not be the same for each person, and they may even change from one treatment session to the next.

Surgery

Surgery to remove the adrenal gland (adrenalectomy) is often used to treat adrenocortical carcinoma. Sometimes the nearby lymph nodes are also removed.

Chemotherapy

Chemotherapy uses drugs to kill and control cancer cells. Learn more about this treatment in our introduction to chemotherapy video.

Radiation therapy

Radiation therapy uses high-energy rays to kill cancer cells. Radiation therapy only affects cells in the treated area.

There are two types of radiation therapy that doctors may use to treat adrenocortical carcinoma:

• External radiation: The radiation comes from a large machine outside the body. The machine aims beams of radiation at the cancer. Patients go to a hospital or clinic for treatment. This type of treatment uses computers to closely target the cancer, which protects healthy tissue near the adrenal glands.
• Internal radiation therapy (brachytherapy): The radiation comes from radioactive material usually contained in very small implants called seeds. The seeds are put near or directly into the cancer, using thin plastic tubes. Brachytherapy is not commonly used to treat adrenocortical carcinoma.

Clinical trials

These studies discover and evaluate new and improved cancer treatments. Patients are encouraged to talk with their doctors about participating in a clinical trial or any questions regarding research studies. For more information, also visit HCI's clinical trials website.

Support

When you or someone you love is diagnosed with cancer, concerns about treatments and side effects, hospital stays, and medical bills are common. You may also worry about caring for your family, work, or normal daily life.

There are several places you can go for support:

• Your health care team can answer your questions and talk to you about your concerns. They can help you with any side effects and keep you informed of all your treatments, test results, and future doctor visits.
• The Cancer Learning Center has hundreds of free brochures and over 3,000 books, DVDs, and CDs available for checkout.You can browse the library, perform Internet research, or talk with a cancer information specialist. Call 801-581-6365 or toll free 1-888-424-2100, or e-mail This e-mail address is being protected from spambots. You need JavaScript enabled to view it .
• Our Patient and Family Support Services offer emotional support and resources for coping with cancer and its impact on daily life to HCI patients and their families.
• The Linda B. and Robert B. Wiggins Wellness-Survivorship Center offers many programs to increase the quality of life and well-being of HCI patients and their families.