Learn About Adrenal Cancer

In order to understand this cancer, it's important to learn about the adrenal gland, in general.

What Are the Adrenal Glands?

Each person is usually born with two adrenal glands. The adrenals are paired, goldenrod-yellow colored glands that are situated behind the organs of the gastrointestinal tract next to the spine and just above the kidneys in a space called the retroperitoneum.

The two adrenal glands are responsible for making hormones -- substances that make other cells in the body respond in various ways. These hormones enable the body to respond to stress, regulate blood pressure and the amount of fluid in the body, and are involved in the regulation of electrolytes such as sodium and potassium.

The hormones produced by the adrenal glands include aldosterone, cortisol, epinephrine and norepinephrine. The adrenal glands also make small amounts of the sex hormones testosterone and estrogen. The ovaries and testicles make the majority of sex hormones. When certain cells in the adrenal glands produce too much of any one of the hormones, abnormalities can result which may affect many organ systems of the body. Disturbances in the amount of hormone produced can be the result of all cells in the gland producing too much hormone (hyperplasia) or be the result of a few cells, a nodule or mass within an otherwise normal gland, producing too much hormone.

Adrenal Cancer: Disease Overview

Adrenal tumors are classified by their growth characteristics:
  • Adrenal adenoma - adrenal tumor that is benign
  • Adrenal carcinoma - adrenal tumor that is malignant (cancer)

Adrenal cancer (adrenocortical carcinoma or ACC) is an extremely rare cancer which occurs in 1-2 people per million people in the population. Approximately 600 patients are diagnosed with adrenal cancer each year in the United States.

At the University of Michigan, we are constantly making progress and strive to improve outcomes for our patients.

For those patients diagnosed with a disease as rare as adrenal cancer, it is important to seek medical advice at a center which specializes in this particular type of cancer. Despite increased utilization of imaging studies, ACC is not being identified at earlier stages in the United States. For various reasons, patients with ACC do not usually present for medical evaluation until late in the disease process. In adults, adrenal cancer often has no obvious symptoms, which makes it difficult to diagnose in its early stages. Those patients who have tumors that produce excess adrenal hormones tend to be diagnosed earlier in the disease process. The most common hormone produced by adrenal cancers is cortisol. This may lead to Cushing’s syndrome. Testosterone is another hormone commonly secreted by adrenal cancers. This may lead to development of a virilizing syndrome. Aldosterone producing adrenal cancers are extremely rare but do occur and can lead to the development of Conn syndrome

Unfortunately, the majority of patients with ACC present with advanced disease, and many are diagnosed at a point when surgery is no longer feasible. These patients are treated with medications to help minimize (palliate) any symptoms they might have, and chemotherapy or radiation therapy may be offered. The Endocrine Oncology Clinic offers a multidisciplinary approach with expertise in endocrinology, endocrine surgery, chemotherapy and radiation therapy to offer patients the highest standard of care. In addition, we work very closely with the University of Michigan Phase I trial center to offer our patients the option of participating in a clinical study using medications that result from decades of adrenal cancer research at the University of Michigan and other institutions.

For those patients who are diagnosed with adrenal cancer at a point in time where surgery is possible, complete surgical resection of all tumor currently provides the only opportunity for cure or long-term survival from ACC and is the treatment of choice. However, surgery with curative intent is not the only indication for surgical intervention in the patient diagnosed with ACC. In those patients with tumors producing excess hormone and suffer severe hypertension, hypokalemia, or severe effects from steroid (cortisol) excess which cannot easily be controlled with medication, surgery may be worthwhile to help decrease the symptoms of hormone excess even if distant metastatic disease is present.

In the United States, forty-five percent of adrenalectomies for ACC are performed in community hospitals, 30% in academic centers, and only 15% in hospitals designated as a National Cancer Institute (NCI) designated cancer center. The University of Michigan Health System is an NCI designated cancer center. Overall there is good evidence that treatment at specialized centers improves patient outcome in terms of cure and survival.

Adrenocortical cancers are often aggressive and can spread to other parts of the body (metastasize) rapidly. The adrenal gland is one of the most vascular organs in the body, with many blood vessels that can carry malignant cells throughout the body. Because the adrenal gland resides behind the intestines and other abdominal organs, it can grow to a very large size before causing any sort of local compressive symptoms or pain. This explains why it is difficult to identify these tumors early on in the disease process, especially if they do not happen to produce any excess amount of adrenal hormones. Unfortunately, symptoms due to adrenal hormone secretion, such as high blood pressure, weight gain, and diabetes are common conditions in middle-aged adults, so the possible connection with an adrenal tumor is often overlooked. The speed of onset of these signs and symptoms may in retrospect be helpful in determining when the tumor started growing. If these conditions develop rapidly, they can be warning signs of adrenal cancer; however, if the adrenal tumor is slow-growing and non-functional (meaning it does not produce excess adrenal hormones), there are usually no symptoms noted in early stages of the disease. As the tumor grows, it may begin to cause local symptoms as it compresses or invades into other organs. In many cases, imaging studies (CT scans) are obtained for vague symptoms that most physicians think are related to a different organ (stomach, gallbladder, pancreas, colon, chronic low back pain) or disease process.

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