Adrenocortical Carcinoma

Adrenocortical carcinoma is also called cancer of the adrenal cortex. There are two adrenal glands in your body, one on top of each kidney. Each adrenal gland has two parts: the center of the adrenal gland is the adrenal medulla and the outer layer is the adrenal cortex, where adrenocortical carcinoma develops.

The adrenal cortex makes important hormones that:

• Balance the water and salt in the body
• Help control the body's use of protein, fat and carbohydrates
• Help keep blood pressure normal
• Cause the body to have masculine or feminine characteristics

A tumor of the adrenal cortex may be functioning (makes more hormones than normal) or nonfunctioning (does not make more hormones than normal). Most adrenocortical tumors are functioning.

What Causes Adrenocortical Carcinoma?

As a type of cancer, adrenocortical carcinoma currently has no known cause. There are risk factors, but having a risk factor does not automatically mean that you will get cancer.

Having certain genetic conditions increase the risk of developing adrenocortical carcinoma, such as:

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

What Are Symptoms of Adrenocortical Carcinoma?

Whether you have a functioning or nonfunctioning adrenocortical tumor will determine what type of symptoms you have; a nonfunctioning tumor may not cause any signs or symptoms in the early stages.

General symptoms of adrenocortical carcinoma include:

• A lump in the abdomen
• Pain the abdomen or back
• A feeling of fullness in the abdomen

A functioning adrenocortical tumor makes too much of one of the following hormones:

• Cortisol
• Aldosterone
• Testosterone
• Estrogen 

Too much cortisol may cause:

• Weight gain in the face, neck and trunk of the body and thin arms and legs
• Growth of fine hair on the face, upper back or arms
• A round, red full face
• A lump of fat on the back of the neck 
• A deepening of the voice and swelling of the sex organs or breasts in both males and females
• Muscle weakness
• High blood sugar
• High blood pressure

Too much aldosterone may cause:

• High blood pressure
• Muscle weakness or cramps
• Frequent urination
• Feeling thirsty

Too much testosterone in women may cause:

• Growth of fine hair on the face, upper back or arms
• Acne
• Balding
• A deepening of the voice
• No menstrual periods

Men who make too much testosterone do not usually have signs or symptoms.

Too much estrogen in women may cause:

• Irregular menstrual periods in women who have not gone through menopause
• Vaginal bleeding in women who have gone through menopause
• Weight gain

Too much estrogen in men may cause:

• Growth of breast tissue
• Lower sex drive
• Impotence

How Is Adrenocortical Carcinoma Diagnosed?

A physical exam, review of your medical history, imaging studies and tests can be used to examine the blood and urine to detect and diagnose adrenocortical carcinoma.

These tests include:

• 24-hour urine test
• Low-dose dexamethasone suppression test
• High-dose dexamethasone suppression test
• Blood chemistry study
• CT scan
• MRI (magnetic resonance imaging)
• Adrenal angiography
• Adrenal venography
• PET scan
• MIBG scan
• Biopsy

How Is Adrenocortical Carcinoma Treated?

There are different types of treatment for patients with adrenocortical carcinoma. Standard treatment options are:

• Surgery
• Radiation therapy
• Chemotherapy

New types of treatment, like biological and targeted therapy, are being tested in clinical trials.