Adrenal cancer, or adrenocortical carcinoma, typically occurs in adults, although it can also occur in children. Only a minority of adrenal cancers are confined to the adrenal gland at the time of diagnosis, and that’s mainly because they are typically not diagnosed early on. If they are caught early on, they are potentially curable through surgery. When the adrenal cancer has grown beyond the adrenal gland or spread to other parts of the body, as it has in the majority of cases at diagnosis, it can be more difficult to treat.

In contrast to many other cancers, there is no effective screening test (like a mammography or colonoscopy) to find adrenal cancer. Further, most doctors in primary care, internal medicine or surgery have never met an adrenal cancer patient. Even doctors who specialize in endocrinology may have gone through their entire career without meeting an adrenal cancer patient. Although there is no recommended screening test for adrenal cancer, if you have ever undergone a CT or MRI of the abdomen (belly), the adrenal glands are included in the scan, and should be evaluated. For many patients, it is helpful to track down any such scans.

There are very few doctors who have significant experience and expertise in adrenal cancer, making it difficult to diagnose and treat.

So, how is adrenal cancer diagnosed?

About half of adrenal cancers are found because the overproduction of hormones causes symptoms, which then prompts patients to seek medical attention. Most of the others are found because of pain or pressure from a large tumor, or by chance on a scan. More on the top symptoms of adrenal cancer.

How is adrenal cancer diagnosed: Through symptoms and bodily changes

Many patients with adrenal cancer present with pain in the abdomen. In fact, many of those who do not present with symptoms from the overproduction of hormones associated with adrenal cancer will experience pain or discomfort in the abdomen and seek medical attention.

Additionally, adrenal cancer is often diagnosed when patients go to their doctor for some sort of bodily change. In adrenal cancer these changes often come on quickly (over weeks to months), and a rapid onset is a warning sign. For example, when excess female hormones are produced in a male it can lead to the development of enlarged breasts (gynecomastia) and excess male hormone production in a female can lead to deeper voice or excess body hair.

Hormone overproduction problems are noted below:

• Hypercortisolism / Cushing's syndrome (excess cortisol produced)
• Adrenal sex hormone excess (excess sex-steroids produced)
• Virilization (acquisition of male traits in a female because of excess testosterone production)
• Feminization (acquisition of female traits in a male because of excess estrogen production)
• Precocious puberty (puberty occurring too early because of excess sex steroids produced)
• Primary aldosteronism / Conn's syndrome (excess aldosterone leading to hypertension and sometimes low potassium)

How is adrenal cancer diagnosed: Through blood and urine tests

Blood and urine tests to measure the adrenal hormones in circulation should be part of the initial evaluation. Since most of these cancers make too much of one or more adrenal hormones, this is an obvious place to begin.

The challenge is that many non-cancerous (benign) adrenal tumors may also secrete too much hormone. This means that even though we can easily demonstrate the presence of an adrenal tumor that is secreting too much hormone, it’s more difficult to distinguish between benign and malignant tumors. Generally, extremely high levels are more common with malignant tumors.

Not all adrenal cancer over produces these hormones, and in those cases, imaging can be helpful in the diagnosis of adrenal cancer.

How is adrenal cancer diagnosed: Through imaging (CT scans, MRI scans)

Imaging scans (such as CT and MRI) play an important role in the diagnosis of adrenal cancers. Further, they are crucial in determining the type of treatment that will be necessary for the patient. Occasionally, other scans such as PET in addition to CT and MRI scans are also very important. They provide overlapping information, so not every patient will need to do all imaging tests. Occasionally, certain cases and patients will require both.

Figure 1. CT scan demonstrating a left adrenal cancer (white arrow) as well as gross pathology proving adrenal cancer (bottom left) after it was removed.

The tricky part is that small adrenal tumors are quite common, and therefore often overlooked in imaging. Unfortunately, many doctors believe that all small adrenal tumors are benign or not important. In fact, many of our adrenal cancer patients were diagnosed with a small adrenal mass in the past but none of their doctors took it seriously. They did not have a follow up scan and they did not have their adrenal hormone levels checked. This is extremely problematic, because we know that if adrenal cancers are not diagnosed early, they can spread to other organs, which makes them much more difficult to treat and cure.

If you ever had an adrenal mass diagnosed on an imaging scan, it is very important you have a follow up to make sure it has not grown and is not overproducing any of the adrenal hormones which are worrisome for cancer.

Figure 2. Two CT scans about 20 months apart in the same patient. Unfortunately, the small tumor on the right adrenal gland (A) was never addressed or diagnosed. It grew to the large right adrenal cancer (B). Although the patient was able to have successful surgery, the patient succumbed to stage 4 metastatic adrenal cancer within 1 year.

How is adrenal cancer diagnosed: Research and collaboration

International efforts are underway to improve care for this rare cancer, and Dr. Carling takes part in the work to enhance collaboration of experts and understand the basis and molecular genetics of adrenal cancer formation. Genetic studies have identified factors that may be used in diagnosing adrenal cancer, as well as to predict treatment response.

Dr. Carling and the Carling Adrenal Center created this website to educate both patients and health care providers about adrenal cancer and how to diagnose adrenal cancer. We strongly believe that educated patients are much better suited to face the challenges of overcoming adrenal cancer.

How is adrenal cancer diagnosed: Seek an expert on adrenal cancer

If you have any symptoms of adrenal cancer, have ever been diagnosed with an adrenal tumor or mass through imaging, or suffer from overproduction of the adrenal hormones mentioned above, you should seek expert evaluation.

Dr. Carling is one of the world’s most experienced adrenal surgeons. To become Dr. Carling’s patient, fill out the new patient form and he’ll be in touch with you shortly to discuss the details of your case.

Additional Resources: 

• Learn more about the Carling Adrenal Center
• Learn more about Dr. Tobias Carling
• Learn more about our sister surgeons at the Norman Parathyroid Center, Clayman Thyroid Center and Scarless Thyroid Surgery Center
• Learn more about the Hospital for Endocrine Surgery