Adrenal cancer is a condition that occurs when abnormal cells form in or travel to the adrenal glands. 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. It generally appears as a tumor.

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.

If you have cancer in the adrenal glands, but it didn’t originate there, it’s not considered an adrenal cortical carcinoma. Cancers of the breast, stomach, kidney, skin, and lymphoma are most likely to spread to the adrenal glands.

Benign adenomas

Benign adenomas are relatively small, usually less than 2 inches in diameter. Most people with this type of tumor have no symptoms. These tumors usually occur on only one adrenal gland, but they can appear on both glands in rare instances.

Adrenal cortical carcinomas

Adrenal cortical carcinomas are usually much larger than benign adenomas. If a tumor is more than 2 inches in diameter, it’s more likely to be cancerous. Sometimes, they can grow large enough to press on your organs, leading to more symptoms. They can also sometimes produce hormones that cause changes in the body.

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:

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.

These include:

  • 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.

Smoking likely also increases the risk of adrenal cancer, but there’s no conclusive proof yet.

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:

Early treatment can sometimes cure adrenal cancer. There are currently three major types of standard treatment for adrenal cancer:

Surgery

Your doctor may recommend a procedure called an adrenalectomy, which involves removing the adrenal gland. If the cancer has spread to other parts of the body, your surgeon may also remove nearby lymph nodes and tissue.

Radiation therapy

Radiation therapy uses high-energy X-rays to kill cancer cells and stop new cancer cells from growing.

Chemotherapy

Depending on the stage of your cancer, you may need to undergo chemotherapy. This form of cancer drug therapy helps stop the growth of cancer cells. Chemotherapy can be administered orally or injected into a vein or muscle.

Your doctor may combine chemotherapy with other types of cancer treatments.

Other treatments

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.

If you develop adrenal cancer, a team of doctors will work with you to coordinate your care. Follow-up appointments with your doctors are important if you’ve had adrenal tumors in the past. Adrenal cancer can come back at any time, so it’s important to stay in close contact with your medical team.