I have an adrenal tumor. Now what?

Consider this scenario: A retired school teacher begins to lose weight, has no appetite, starts feeling weak and experiences episodes of severe headaches.  A trip to an emergency room shows she has a dangerously elevated blood pressure level.  A series of subsequent tests reveal a mass on her right adrenal gland.  She is diagnosed with an adrenal tumor known as pheochromocytoma.  A team of doctors is assembled.  Special medications are given to block the adrenaline hormone that the tumor is secreting and the patient undergoes a surgery known as laparoscopic adrenalectomy.  After a brief hospital stay, she recovers fully.  With her symptoms resolved, she regains weight and moves on with her life.

Diagnosis and treatment of patients with adrenal tumors can be one of the most gratifying and rewarding clinical encounters of modern medicine.  On average, 5 percent of people harbor adrenal lesions, and it is important that an experienced team of specialists helps navigate the often complex waters of this diagnosis.

Here are the top questions regarding adrenal tumors answered.

What is the adrenal gland?
Adrenal glands come in pairs, located above the two kidneys in an area of the body behind the bowel sac known as the retroperitoneum.  These small organs serve a critical role in keeping the body's physiological balance. The loss of both glands requires medications to replace critical hormones. The glands tend to be hidden in the deepest part of the body and are often surrounded by sticky fatty tissue.  In fact, this is the reason why safe adrenal surgery requires significant experience and expertise.

Is an adrenal tumor dangerous?
The vast majority of adrenal tumors are benign — known as adenomas. Cancer can occur in the adrenal gland; however, adrenocortical carcinoma is truly a one in a million tumor with only about 300 people being diagnosed in the United States each year.  Therefore, if you or a loved one is told they have an adrenal tumor, it is very unlikely to be cancer.

Although most adrenal tumors are benign, not all of them are harmless. Up to 10 percent of adrenal masses can produce too much adrenal hormone, which can have ill effects on the rest of the body. High blood pressure, increased risks of heart disease and stroke, weight gain for some patients and weight loss for others can all stem from adrenal tumors.  Conditions known as Cushing Syndrome, Conn Syndrome, and Pheochromocytoma can all stem from abnormalities in the adrenal gland.

I have an adrenal tumor. Now what?
On average, adrenal tumors are approximately 3cm in diameter when discovered.  How the adrenal tumor looks on the computed tomography or magnetic resonance imaging scan is important and helps doctors make recommendations for treatment.   When the tumors lack cellular fat content and grow over 4cm, doctors may recommend tumor removal.  Nevertheless, all adrenal tumors more than 1cm in size, regardless of imaging characteristics, deserve a metabolic work-up, which involves a series of blood or urine tests. Testing for hypersecretion of a hormone known as cortisol and for metabolites of substances known as catecholamines should be performed.  Generally doctors perform what's known as a low-dose dexamethasone suppression test and check the blood or urine for substances called metanephrines.  Patients with a history of elevated blood pressure should also be tested for elevated levels of the hormone aldosterone.

I need an adrenal surgery. What should I expect?
It is important to know that the vast majority of adrenal tumors will never require surgery.  Nevertheless, those tumors that look worrisome on imaging and/or get too large or those that secrete excess adrenal hormones should be removed. Surgery to remove an adrenal gland is called adrenalectomy.  Sometimes, especially in patients who are at risk of developing more than one adrenal tumor, only a portion of the adrenal gland is removed – a procedure known as partial adrenalectomy.  For patient's diagnosed with pheochromocytoma, appropriate hormonal blockade is important before surgery is undertaken.

The advent of minimally invasive surgery (laparoscopic / robotic surgery) has allowed surgeons to significantly reduce the discomfort and length of recovery after adrenalectomy.  Using these modern approaches, the adrenal surgeon enters the body with instruments and cameras through keyhole incisions, allowing for a much quicker recovery. However, experienced adrenal surgeons still employ traditional open surgery selectively, since it is believed to be a safer operation for tumors that are likely to prove cancerous.

Ultimately, diagnosis of an adrenal mass often comes as a surprise, but most tumors don't need treatment.  Nevertheless, appropriate evaluation and management of these masses requires deep and nuanced expertise.

Alexander Kutikov, MD, FACS is a professor and chief of urologic surgical oncology at Fox Chase Cancer Center.