Adrenal Cancer: An Overview of Adrenal Cancer

What is Adrenal Cancer?

A typical 10 cm adrenal cancer demonstrating irregular borders, invasion, and areas of necrosis (dead cells) and hemorrhage (bleeding).

Adrenal cancer (or adrenocortical carcinoma; ACC) is a relatively rare but aggressive cancer. The only potential cure is complete surgical removal. Adrenal cancer usually occurs in adults, and the median age at diagnosis is 44 years, but adrenal cancer can occur in children, as well as in the elderly. Although potentially curable early on, only 30% of these cancers are confined to the adrenal gland at the time of diagnosis. That means that in 70%, the adrenal cancer has already spread to other parts of the body (abdominal cavity, lungs, liver, and bone) by the time the cancer is detected. Because these tumors tend to be found years after they began growing, they can invade nearby organs, spread to distant organs (metastasize) and cause numerous changes (signs and symptoms) in the body because of the excess hormones they produce. NOTE: When we talk about adrenal cancer, we mean adrenocortical carcinoma, which arises from the adrenal cortex. Cancers arising from the adrenal medulla are called malignant pheochromocytoma. Read about malignant pheochromocytoma.

Adrenocortical carcinoma is an aggressive with poor prognosis. Again, complete surgical resection offers the sole chance for potential cure. You need the most experienced surgeons in the world operating on you or your loved ones if you have adrenal cancer. Given that adrenal cancer is rare, there has been international efforts to improve our care of adrenal cancer patients. Dr. Carling is very much part of these efforts to enhance collaboration and understanding the basis (molecular genetics) of adrenal cancer formation. These scientific genetic studies have identified factors that may be used in the diagnosis, as well as predict treatment response. The Carling Adrenal Center created this website to educate both patients and health care providers including other doctors about adrenal cancer. We strongly believe that educated patients are much better suited to face the challenges of overcoming adrenal cancer.

What are the characteristics of Adrenal Cancer?

MRI showing an adrenal cancer (arrow) arising from the right adrenal gland. Note the heterogeneity and irregular borders. This tumor overproduced cortisol causing Cushing's syndrome.

• Typically adrenocortical carcinoma is an aggressive cancer
• Most (~60%) are found because excess hormone production causes symptoms which prompt patients to seek medical attention
• Most (60-80%) secrete high amounts of one or more adrenal hormones
• Many will present with pain in the abdomen and flank (nearly all that do not present with symptoms of hormone excess will seek medical attention because of pain)
• Spread to distant organs (metastasis) occurs most commonly to the abdominal cavity, lungs, liver, and bone
• The initial evaluation should include blood tests to measure adrenal hormones in the circulation (blood and urine hormone tests). Since most of these cancers make too much hormone (cortisol, testosterone, estrogen, other androgens, DHEAS, aldosterone, etc.), this is an obvious place to start. Keep in mind, however, that most non-cancerous tumors of the adrenal glands (benign adenomas and hyperplasia) may also secrete too much hormones. Therefore, demonstrating overproduction of adrenal hormones helps establish the presence of an adrenal tumor, yet it does not always help distinguish between benign and malignant (cancerous) tumors. Extremely high levels, however, are more commonly produced by malignant tumors.
• X-ray tests (scans) play a central role in the diagnosis of adrenal cancers, and undoubtedly will play a central role in determining the type of treatment planned. Computed tomography (CT scans) and/or magnetic resonance imaging (MRI) are the two central tests in this regard. They give overlapping information, so that all persons do not need both tests, but occasionally the situation will dictate that both are obtained.
CT showing liver metastases (arrows) from an adrenal cancer arising in the right left gland. This tumor overproduced estrogen causing feminization (acquisition of female traits in a male).
• Many patients will seek medical attention with some sort of bodily change which typically comes on quite slowly (usually over 1 to 3 years). When excess female hormones are produced in a female it can be hard to detect, except at extremes of age such as early puberty in a child, or the return of vaginal bleeding in a post-menopausal woman. The same is true for excess testosterone in a male. The opposite, however, will often make the presentation obvious, such as when a woman begins to develop male characteristics (deeper voice, excess body hair) or when a man begins to develop enlarged breasts (gynecomastia). Some of these hormone overproduction problems have specific names:
• Hypercortisolism; Cushing's syndrome (excess cortisol produced)
• Adrenogenital syndrome (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)
• Hyperaldosteronism; Conn's syndrome (excess aldosterone leading to hypertension and low potassium)

Fortunately adrenocortical cancer (ACC) and metastasis to the adrenal gland are relatively rare. We have hundreds of pages of information and photos and graphics to help you and your doctor understand every adrenal problem, including cancerous ones. Please use the navigation tools or even the "search" button to locate anything you want to know about the adrenal glands.