Adrenal cancer is a rare cancer that begins in one or both of the small, triangular glands (adrenal glands) located on top of your kidneys. Adrenal glands produce hormones that give instructions to virtually every organ and tissue in your body. Adrenal cancer, also called adrenocortical cancer, can occur at any age. But it’s most likely to affect children younger than 5 and adults in their 40s and 50s. When adrenal cancer is found early, there is a chance for cure. But if the cancer has spread to areas beyond the adrenal glands, cure becomes less likely. Treatment can be used to delay progression or recurrence. Most growths that form in the adrenal glands are noncancerous (benign). Benign adrenal tumors, such as adenoma or pheochromocytoma, also can develop in the adrenal glands.

Symptoms

Signs and symptoms of adrenal cancer include:

• Weight gain
• Muscle weakness
• Pink or purple stretch marks on the skin
• Hormone changes in women that might cause excess facial hair, hair loss on the head and irregular periods
• Hormone changes in men that might cause enlarged breast tissue and shrinking testicles
• Nausea
• Vomiting
• Abdominal bloating
• Back pain
• Fever
• Loss of appetite
• Loss of weight without trying

Causes

It’s not clear what causes adrenal cancer. Adrenal cancer forms when something creates changes (mutations) in the DNA of an adrenal gland cell. A cell’s DNA contains the instructions that tell a cell what to do. The mutations can tell the cell to multiply uncontrollably and to continue living when healthy cells would die. When this happens, the abnormal cells accumulate and form a tumor. The tumor cells can break away and spread (metastasize) to other parts of the body.

Risk factors

Adrenal cancer happens more often in people with inherited syndromes that increase the risk of certain cancers. These inherited syndromes include:

• Beckwith-Wiedemann syndrome
• Carney complex
• Li-Fraumeni syndrome
• Lynch syndrome
• Multiple endocrine neoplasia, type 1 (MEN 1)

Diagnosis

Tests and procedures used to diagnose adrenal cancer include:

• Blood and urine tests. Laboratory tests of your blood and urine may reveal unusual levels of hormones produced by the adrenal glands, including cortisol, aldosterone and androgens.
• Imaging tests. Your doctor may recommend CT, MRI or positron emission tomography (PET) scans to better understand any growths on your adrenal glands and to see if cancer has spread to other areas of your body, such as your lungs or your liver.
• Laboratory analysis of your adrenal gland. If your doctor suspects you may have adrenal cancer, he or she may recommend removing the affected adrenal gland. The gland is analyzed in a laboratory by a doctor who studies body tissues (pathologist). This analysis can confirm whether you have cancer and exactly what types of cells are involved.

Treatment

Adrenal cancer treatment usually involves surgery to remove all of the cancer. Other treatments might be used to prevent the cancer from coming back or if surgery isn’t an option.

Surgery

The goal of surgery is to remove the entire adrenal cancer. To achieve this, doctors must remove the all of the affected adrenal gland (adrenalectomy). If surgeons find evidence that cancer has spread to nearby structures, such as the liver or kidney, parts or all of those organs might also be removed during the operation.

Medication to reduce the risk of recurrence

An older drug that has been used to treat advanced adrenal cancer has shown promise in delaying the recurrence of the disease after surgery. Mitotane (Lysodren) may be recommended after surgery for people with a high risk of cancer recurrence. Research into mitotane for this use is ongoing.

Radiation therapy

Radiation therapy uses high-powered beams of energy, such as X-rays and protons, to kill cancer cells. Radiation therapy is sometimes used after adrenal cancer surgery to kill any cells that might remain. It can also help reduce pain and other symptoms of cancer that has spread to other parts of the body, such as the bone.

Chemotherapy

Chemotherapy is a drug treatment that uses chemicals to kill cancer cells. For adrenal cancers that can’t be removed with surgery or that return after initial treatments, chemotherapy may be an option to slow the progression of the cancer.

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