Adrenocortical Carcinoma

What is cancer of the adrenal cortex?

Cancer of the adrenal cortex, a rare cancer, is a disease in which cancer (malignant) cells are found in the adrenal cortex, which is the outside layer of the adrenal gland. Cancer of the adrenal cortex is also called adrenocortical carcinoma. There are two adrenal glands, one above each kidney in the back of the upper abdomen. The adrenal glands are also called the suprarenal glands. The inside layer of the adrenal gland is called the adrenal medulla. Cancer that starts in the adrenal medulla is called phaeochromocytoma and is discussed in a separate patient information summary.

The cells in the adrenal cortex make important hormones that help the body work properly. When cells in the adrenal cortex become cancerous, they may make too much of one or more hormones, which can cause symptoms such as high blood pressure, weakening of the bones, or diabetes. If male or female hormones are affected, the body may go through changes such as a deepening of the voice, growing hair on the face, swelling of the sex organs, or swelling of the breasts. Cancers that make hormones are called functioning tumours. Many cancers of the adrenal cortex do not make extra hormones and are called nonfunctioning tumours.

A doctor should be seen if the following symptoms appear and won’t go away:

* pain in the abdomen,
* loss of weight without dieting, or
* weakness.

If there is a functioning tumour, there may be symptoms or signs caused by too many hormones.

If there are symptoms, a doctor will order blood and urine tests to see whether the amounts of hormones in the body are normal. A doctor may also order a computed tomography scan of the abdomen, a special x-ray that uses a computer to make a picture of the inside of the abdomen. Other special x-rays may also be done to tell what kind of tumour is present.

The chance of recovery (prognosis) depends on how far the cancer has spread (stage) and on whether a doctor was able to surgically remove all of the cancer.

Stage Explanation

Stages of cancer of the adrenal cortex

Once cancer of the adrenal cortex has been found, more tests will be done to see how far the cancer has spread. This is called staging. A doctor needs to know the stage of the cancer to plan treatment. The following stages are used for cancer of the adrenal cortex:
Stage I

The cancer is less than 5 centimeters (less than 2 inches) and has not spread into tissues around the adrenal gland.
Stage II

The cancer is more than 5 centimeters (greater than 2 inches) and has not spread into tissues around the adrenal gland.
Stage III

The cancer has spread into tissues around the adrenal gland or has spread to the lymph nodes around the adrenal gland. Lymph nodes are part of the lymph system and are small, bean shaped organs that make and store infection-fighting cells.
Stage IV

The cancer has spread to tissues or organs in the area and to lymph nodes around the adrenal cortex, or the cancer has spread to other parts of the body.
Recurrent

The cancer has come back (recurred) after it has been treated. It may come back in the adrenal cortex or in another part of the body.

Treatment Option Overview

How cancer of the adrenal cortex is treated

There are treatments for all patients with cancer of the adrenal cortex. Three kinds of treatment are used:

* Surgery (taking out the cancer).
* Chemotherapy (using drugs to kill cancer cells).
* Radiation therapy (using high-dose x-rays or other high-energy rays to kill cancer cells).

A doctor may take out the adrenal gland in an operation called an adrenalectomy. Tissues around the adrenal glands that contain cancer may be removed. Lymph nodes in the area may also be removed (lymph node dissection).

Chemotherapy uses drugs to kill cancer cells. Chemotherapy may be taken by pill, or it may be put into the body by a needle in a vein or muscle. Chemotherapy is called a systemic treatment because the drug enters the bloodstream, travels through the body, and kills cancer cells throughout the body.

Radiation therapy uses high-energy x-rays to kill cancer cells and shrink tumours. Radiation for cancer of the adrenal cortex usually comes from a machine outside the body (external radiation therapy).

Besides treatment for cancer (chemotherapy, radiation therapy, and/or surgery), a patient may also receive therapy to prevent or treat symptoms caused by the extra hormones that are made by the cancer.
Treatment by stage

Treatment depends on how far the cancer has spread, and a patient’s age and overall health.

Standard treatment may be considered because of its effectiveness in past studies, or participation in a clinical trial may be considered. Not all patients are cured with standard therapy, and some standard treatments may have more side effects than are desired. For these reasons, clinical trials are designed to find better ways to treat cancer patients and are based on the most up-to-date information. Clinical trials are ongoing in some parts of the country for patients with cancer of the adrenal cortex. For more information, call the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237); TTY at 1-800-332-8615.

Stage I Adrenocortical Carcinoma

Treatment will probably be surgery to remove the cancer.

Stage II Adrenocortical Carcinoma

Treatment will probably be surgery to remove the cancer. Clinical trials are testing new treatments.

Stage III Adrenocortical Carcinoma

Treatment may be one of the following:

1. Surgery to remove the cancer. Lymph nodes in the area may also be removed (lymph node dissection).
2. A clinical trial of radiation therapy.
3. A clinical trial of chemotherapy if the size of the tumour can be measured with x-rays and/or if the tumour is making hormones.

Stage IV Adrenocortical Carcinoma

Treatment may be one of the following:

1. Chemotherapy. Clinical trials are testing new drugs.
2. Radiation therapy to bones where the cancer has spread.
3. Surgery to remove the cancer in places where it has spread.

Recurrent Adrenocortical Carcinoma

Treatment depends on many factors, including where the cancer came back and what treatment has already been received. In some cases, surgery can be effective in decreasing the symptoms of the disease by removing some of the tumour. Clinical trials are testing new treatments.