A medical illustration of the adrenal glands, which sit on top of the kidneys
A medical illustration of the adrenal glands, which sit on top of the kidneys
A medical illustration of the adrenal glands, which sit on top of the kidneys

Adrenocortical Carcinoma

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Adrenocortical carcinoma is a rare disease that can affect children and middle-aged adults. Females are more frequently diagnosed with adrenocortical carcinoma than males. Endocrinologist Amir Hamrahian, M.D., and endocrine surgeon Lilah Morris-Wiseman, M.D., explain the diagnosis, causes and treatment for adrenocortical carcinoma.

What You Need to Know

  • Adrenocortical carcinoma forms on the outermost part of the adrenal gland.
  • Symptoms of adrenal cancer might include weight gain, muscle weakness, trouble sleeping, deepening voice and increased hair growth, usually on the face (in women), pain in the abdomen or lower back, weight loss or loss of appetite.
  • Surgical removal (adrenalectomy) is the main treatment for adrenal cancer.
  • Periodic follow-up after treatment is necessary to monitor your health and maintain healthy adrenal hormone levels.

What is adrenocortical carcinoma?

Adrenal cancer happens when healthy cells in the adrenal gland mutate and grow out of control. The adrenal gland has an outer cortex and an inner medulla. Cancerous tumors of the outer part of the adrenal gland are called adrenocortical carcinoma.

What causes adrenocortical carcinoma?

Adrenocortical carcinoma typically develops sporadically, without a genetic link. In some cases, this cancer has been associated with genetic conditions, including:

  • Li-Fraumeni syndrome
  • Beckwith-Wiedemann syndrome
  • Multiple endocrine neoplasia type 1 (MEN1)

What are the symptoms of adrenocortical carcinoma?

Often, you may not experience any symptoms. “Many people find out they have it after their doctor does an imaging test, such as a CT scan, or another reason,” says Hamrahian. “In other patients, adrenal cancer makes abnormally high levels of hormones and causes symptoms such as weight gain or loss, muscle weakness, trouble sleeping or a deepening voice. Women may develop facial hair.”

Additional symptoms of adrenocortical carcinoma include pain in the abdomen or lower back and appetite changes.

How is adrenocortical cancer diagnosed?

Tests for adrenal cancer usually include:

  • Blood and urine tests to measure different hormones produced by the adrenal gland
  • Imaging tests such as CT scan and MRI

How is adrenocortical cancer treated?

Your treatment will depend on the stage of your cancer and your medical history.

“Most people with adrenocortical cancer that has not spread are treated with surgery to remove the tumor,” explains Morris-Wiseman. “After surgery, chemotherapy is usually recommended.” If the cancer has already spread, patients are treated with chemotherapy and possibly radiation therapy.

“Because adrenocortical cancer is so rare, patients with this cancer should be treated in a specialized center with surgeons who have expertise in treating it. In our multidisciplinary adrenal center, the surgeons, endocrinologists and oncologists all work together to plan treatment and monitor success.”

Living with adrenal cancer

After treatment, your doctor will continue to monitor your health. Follow-up tests include exams in the office, blood tests and imaging tests. If your adrenocortical carcinoma returns, your doctor may recommend additional surgery, chemotherapy or radiation to treat the cancer.

You might need to take long-term medicines to correct the levels of hormones in your body. To monitor your hormone levels, your doctor will do regular follow-up blood tests.

Roshan Dinparastisaleh, 2021 endocrinology research fellow, prepared this article under the supervision of Amir Hamrahian and Lilah Morris-Wiseman.

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