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Learn About Adrenal Cancer

Introduction   |  Symptoms   |  Survival Rates

In order to understand this cancer, it's important to learn about the adrenal gland, in general. The two adrenal glands are positioned above the kidneys and are responsible for making steroid hormones that enable the body respond to stress (aldosterone, cortisol and adrenaline). They also make a small amount of sex steroids (the major sex steroids testosterone and estrogen are made by the gonads).

Introduction

Adrenal tumors often are characterized by the hormones that they secrete:

• Aldosterone tumor (Conn's Syndrome) = adrenal tumor making aldosterone: often presenting with new hypertension and low potassium . This syndrome is named after the former UMICH endocrinologist who discovered and characterized this syndrome.
  
• Cushing's Syndrome = adrenal tumor making excessive cortisol: often presenting with new hypertension, diabetes, central obesity.
  
• Virilizing adrenal tumor = adrenal tumor making excessive androgens (like testosterone) often presenting with new hirsuitism (hair growth), increased muscle mass, acne and amenorhea (loss of periods) in a female.
  
• Feminizing adrenal tumor = adrenal tumor making excessive estrogens often presenting with new gynecomastia (breast growth in a man) and impotence in a male.
  
• Pheochromocytoma = adrenal tumor making excessive adrenaline often presenting with new hypertension, headaches, sweating, palpitations and panic attacks.

Additionally adrenal tumors are classified by their growth characteristics:

Adrenal adenoma = adrenal tumor that is benign

Adrenal carcinoma = adrenal tumor that is malignant

Adrenocortical carcinoma (ACC) is rare, but very deadly. This type of cancer develops in the outer layer, called the cortex, of the two small adrenal glands that sit on top of each kidney. About 600 new cases are diagnosed each year in the United States, or about one to two cases per million people.

Adrenocortical cancer is most common in either the first or fifth decade of life. Because it produces symptoms in children, pediatric adrenal cancer is often diagnosed and treated at an early stage. Five-year survival rates in children are greater than 50 percent.

Adults rarely have obvious symptoms, so they often are not diagnosed until the cancer is large and has spread to other parts of the body. Although adrenal cancer can be treated in adults, it usually comes back. Once it recurs, it is almost always fatal.

 

Symptoms

In adults, adrenal cancer usually has no obvious symptoms, which makes it very hard to diagnose in its early stages. Adrenocortical carcinomas are aggressive and spread rapidly. The adrenal gland is one of the most vascular organs in the body, with lots of blood vessels that can carry malignant cells throughout the body. Scientists believe metastasis, or the spread of cancer cells to other organs, begins very early in adrenal cancer.

Sometimes a rapidly growing tumor will cause the adrenal gland to make excessive amounts of certain hormones. Too much aldosterol can cause hypertension or high blood pressure. Too much cortisol can cause rapid weight gain, hypertension or diabetes. Too much sex hormone can cause facial hair in women or breast development in men.

Unfortunately, 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 until it's too late. The key is speed of onset. 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 affect hormone production - there are no symptoms in early stages of the disease. This type of tumor is sometimes diagnosed "by accident" if the patient has a CT scan for some other reason.

Because this type of cancer is so rare, physicians don't know much about it. Many patients are referred to the U-M Comprehensive Cancer Center - one of only a few medical centers in the United States recognized as an international center of excellence for the treatment of adrenal cancer.

Scientists in the UMCCC's adrenal cancer program are studying primitive cells, called stem or progenitor cells, found in the outer cortex of the adrenal gland. They believe defects in these stem cells and the genes that regulate them could be the cause of adrenal cancer. Discovering what goes wrong early in the development of adrenal cancer is the vital first step toward finding better treatments and a cure for this fatal disease.

 

Survival Rates

Because adult adrenal cancers are aggressive and almost always diagnosed late, the chances for survival are not good. Survival rates vary with the stage of the tumor.

In stage I disease, the tumor is less than 5 centimeters in diameter (about the size of a dime) and is contained within the adrenal gland. In stage II, the tumor is more than 5 centimeters, but still present only in the adrenal gland. In stage III, the tumor has spread locally to areas near the adrenal gland. In stage IV, cancer has spread to other parts of the body.

In 40 percent of patients diagnosed with stage I adrenocortical carcinoma, the cancer recurs after treatment. For those diagnosed in stage II, the recurrence rate is 60 percent. For stage III, 70 percent to 80 percent of patients experience recurrence. Patients diagnosed in stage IV face a 90 percent or higher rate of recurrence.

After a recurrence of adrenal cancer, virtually all patients die within five years.

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