Adrenocortical Carcinoma

Definition

Adrenocortical carcinoma is cancer that starts in the adrenal cortex. There are 2 adrenal glands above each kidney. They make hormones that control blood pressure and water balance. They also manage the response to stress and infection.

This type of cancer is rare. In most cases, these tumors make too much of certain hormones. This can lead to an imbalance.

Anatomy of the Adrenal Glands

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Causes

Cancer happens when cells divide without control or order. These cells grow together to form a tumor. They can invade and damage nearby tissues. They can also spread to other parts of the body.

It is not clear what causes changes in the cells. It is likely a combination of genes and environment.

Risk Factors

Adrenocortical carcinoma is more common in people who have:

• Close family members with adrenocortical carcinoma
• Certain genetic conditions, such as:
  • Beckwith-Wiedemann syndrome
  • Carney complex
  • Familial adenomatous polyposis (FAP)
  • Li-Fraumeni syndrome
  • Lynch syndrome
  • Multiple endocrine neoplasia (MEN1)
  • Neurofibromatosis Type 1 (NF1)
  • Von Hippel-Lindau (VHL) syndrome.

Symptoms

Most people with adrenocortical carcinoma do not have symptoms. If symptoms appear, they are linked to an imbalance in hormones. Symptoms may include:

• Weight gain in the face (rounding), neck, or mid-body
• Easy bruising
• Emotional changes such as:
  • Irritability
  • Restlessness
  • Depression
• Muscle weakness
• Skin problems such as thinning or acne
• Excess face or body hair in women
• Increase in breast size in men not related to weight gain
• Irregular menstrual periods

The problems depend on which hormone is out of balance.

Diagnosis

Adrenal cancer is often found during routine image testing. The doctor will ask about symptoms and past health. A physical exam will be done. Tests may include:

• Blood tests
• Urine tests
• Hormone testing—to find out what hormone is causing problems
• Imaging tests such as:
  • CT scan
  • MRI scan
  • PET/CT scan
  • X-rays

More tests will be done to find the stage of the cancer. The stage of a tumor is based on its size and how far it has spread.

Treatment

Treatment depends on the stage of the tumor. One or more methods may be used such as:

• Surgery— Adrenalectomy to remove the adrenal tumor. This is often the first treatment if the tumor is causing problems.
• Radiation therapy—Used to shrink any remaining tumors after surgery or if the cancer has spread. It may be:
  • External—radiation aimed at the tumor from a source outside the body
  • Internal—radioactive materials are placed into the body near the cancer cells
• Chemotherapy—Use of drugs to kill cancer cells. The drugs enter the bloodstream and travel throughout the body. It is not given as a cure. It may be used if cancer has spread and surgery is not possible.
• Medicines are used to:
  • Kill cancer cells
  • Balance hormones
  • Manage symptoms caused by hormones

Prevention

There is no way to prevent adrenal cancer since the cause is unknown.