Q: What is adrenal cortex cancer?

A: The adrenal glands sit on top of the kidneys; the outside of the gland is called the cortex, and the inside the medulla. The adrenal glands produce hormones that help regulate:

The stress responses (such as the fight or flight response) with the catecholamines adrenaline (also called epinephrine) and noradrenaline made in the medulla.
Blood pressure/blood electrolytes with mineralocorticoids made in the cortex.
Certain male and female sex hormones (androgens such as testosterone in men or estrogen in women), also made in the cortex.
General metabolism and immune system control with glucocorticoids made in the cortex.

Tumors of the adrenal glands are very common, and are usually benign; they may be found incidentally on a CT or MRI of the patient’s abdomen/pelvis done for other reasons. Benign adrenal tumors are noted in up to 10 percent of people at autopsy. However, about 5 to 10 percent of adrenal masses are cancerous.

Adrenal cortical carcinoma (ACC) is a rare cancerous growth in the cortex of the adrenal gland; there are only one to two cases of this cancer per million Americans diagnosed per year. ACC may have functional cancer cells which produce some of the hormones noted above, or it may be non-functional.

Non-functional ACC may produce symptoms of abdominal pain, abdominal mass and/or fullness. When the ACC cancer cells are functioning cells, the initial symptoms from the disease are often related to hormonal overproduction. This may include:

Increased facial/body hair and/or deepened voice from testosterone (especially noted in women if this hormone is produced).
Breast enlargement from estrogen (especially noted if this hormone is produced in men).
Weight gain and/or high blood pressure from mineralocorticoids.
High blood sugar, high blood pressure, weakness, excessive weight gain, and/or fatty tissue. Deposits (for example in the upper back/neck between the shoulders sometimes referred to as a buffalo hump) from glucocorticoids.

Note that many other conditions can cause excess hormone production, and most of these are non-cancerous, so having excess hormone production is not diagnostic of ACC.

When ACC is suspected, the patient will undergo an imaging test (CT, MRI and/or PET scan) to evaluate for the presence of a mass on the adrenal gland. Specialized blood and urine tests may be done to evaluate for possible hormone overproduction. Microscopic analysis of tissue from a biopsy of any mass identified will help confirm the diagnosis.

If the ACC is functional, treatment for the excess hormone levels, and any complications from that, may be needed. For the cancer itself, the treatment depends on the stage (which is a way of noting how progressed the disease is):

Stage 1 means that the tumor is less than 2 inches in diameter and it appears to be confined to the adrenal gland.
Stage 2 is greater than 2 inches but also thought to be confined to the adrenal gland.
Stage 3 has spread to the nearby fatty tissue and/or lymph nodes.
Stage 4 (also called advanced/metastatic cancer) has spread to other organs or tissues.

ACCs are often very aggressive cancers, and hence many patients initially assessed as having earlier stage disease (including stage 1, 2 or 3) are subsequently found to have distant metastatic disease (stage 4).

Treatment options include surgery (to remove the adrenal gland and surrounding tissue, and possibly to remove cancer in other locations), chemotherapy (mitotane is one medication often used, although ACC is often not very responsive to chemotherapy), radiation treatment, and/or newer treatments (such as agents to help the patient’s own immune system more effectively fight the cancer).

The prognosis for ACC patients depends on the stage of their disease. The overall 5-year survival rates (percentage of patients still alive 5 years after diagnosis) for patients with ACC stage 1, 2, 3 and 4 are less than 85, 65, 40 and 15 percent respectively.

Unfortunately, the aggressiveness of SCC increases the risk of its recurrence after appropriate treatment. Therefore, patients will need to be closely followed. Recommendations of follow up CT or MRI scans every 3 to 6 months for a few years and then every 1 to 2 years are often made. Clinical trials are being performed to hopefully find more efficacious treatments. Patients diagnosed with ACC should speak to their treating clinician to see if enrolling in a clinical trial is right for them.

Jeff Hersh, Ph.D., M.D., can be reached at DrHersh@juno.com