Hyperfunctional Tumors

One common reason a person may need to have an adrenalectomy is a hyperfunctional tumor. This kind of tumor inappropriately overproduces hormones.

There are three main types of hyperfunctional tumors: pheochromocytomas, aldosteronomas, and cortisol-producing adenomas. Each is described in detail below.


1. Pheochromocytoma

This is a type of tumor originating from the adrenal medulla. These tumors secrete excessive amounts of catecholamines, including epinephrine (adrenaline) and norepinephrine.

Signs and symptoms of pheochromocytoma:

  • Severe hypertension (high blood pressure)
  • Tachycardia (increased heart rate)
  • Palpitations
  • Cardiac arrhythmias (irregular heartbeat)
  • Anxiety attacks
  • Weight loss
  • Sweating
  • Headaches

Some statistics:

  • 30% of pheochromocytomas are hereditary (familial).
  • 10% involve both adrenal glands (bilateral).
  • 10% occur outside the adrenal gland (paragangliomas).
  • 10% are malignant (cancerous).

Diagnosis is made by blood and/or urine tests that measure catecholamines and metanephrines. Once the biochemical diagnosis has been made, an imaging study will be used to determine the location of the tumor (i.e., right vs. left adrenal gland, inside vs. outside the adrenal gland). Imaging options include CT, MRI, and MIBG scans.

The treatment for pheochromocytoma is adrenalectomy (removal of the affected adrenal gland). Prior to your operation, you will be placed on an antihypertensive medication for 1-3 weeks. This helps prevent unstable blood pressure during surgery.

Pheochromocytoma scan

A pheochromocytoma is a type of tumor originating in the medulla region of the adrenal gland. In this image, the tumor shows up as a yellow spot. Our hospital uses the latest techniques to combine MIBG scintigraphy and CT scans, resulting in a single, fused image that leads to better diagnosis. Photo courtesy of Dr. Phillip Koo, University of Colorado Hospital.


2. Aldosteronoma (Aldosterone-producing Adrenal Adenoma)

Aldosterone-producing adrenal adenomas (often referred to simply as “aldosteronomas”) are tumors originating from the glomerulosa zone of the adrenal cortex. They produce too much aldosterone, resulting in a condition known as primary hyperaldosteronism.

Signs and symptoms of primary hyperaldosteronism:

  • Hypertension (high blood pressure)
  • Hypokalemia (low potassium levels)
  • Muscle cramping
  • Muscle weakness
  • Polydipsia (increased thirst)
  • Polyuria (increased urinary frequency)
  • Nocturia (increased nighttime urination)

Diagnosis is made by blood tests that measure aldosterone and renin levels.  If these tests are suggestive of disease, a confirmatory test with salt loading will be performed.

Once the diagnosis is established, a CT/MRI adrenal protocol will be performed to determine the location of the tumor. In most cases, adrenal vein sampling will also be performed to verify that the lesion seen on the imaging study is definitely the one causing the hormonal hypersecretion. This test is performed in Interventional Radiology and involves placing a small catheter in a groin vein to measure the aldosterone levels in each adrenal vein.

Note: Another condition that can cause primary hyperaldosteronism is unilateral hyperplasia of the adrenal gland. Adrenal vein sampling, as described above, can be used to determine whether the patient has a unilateral hyperplasia (which can be cured by adrenalectomy) or bilateral hyperplasia (which is best treated with medicine.)


3. Cortisol-producing Adrenal Adenoma

These tumors originate in the fasciculata zone of the adrenal cortex and cause excessive production of cortisol, which leads to hypercortisolism (Cushing Syndrome).

Signs and symptoms of hypercortisolism:

  • Fatigue
  • Depression
  • Hypertension (high blood pressure)
  • Central (abdominal) obesity and general weight gain
  • Glucose intolerance (diabetes)
  • Osteoporosis (weak bones)
  • Muscle weakness
  • Ecchymosis (bruising easily)
  • Acne
  • Hirsuitism (excessive hair
  • Menstrual irregularities

Diagnosis is made with blood, urine, or salivary tests that measure cortisol levels. If these are suggestive of disease, a confirmatory test will be performed (dexamethasone suppression test).

Once the diagnosis is established, a CT/MRI adrenal protocol will be performed to determine the location of the tumor. The adrenal gland where the tumor is located will be surgically removed.

After the operation, you will need to take steroids until the remaining gland restarts its function. (This gland, while capable of producing cortisol, has been "asleep" because the body has been signaling it that cortisol levels were high.)

 


This information is provided by the Department of Surgery at the University of Colorado School of Medicine. It is not intended to replace the medical advice of your doctor or healthcare provider. Please consult your healthcare provider for advice about a specific medical condition.