Cushing´s Syndrome

Cushing's syndrome, less common than the endocrine system diseases discussed above, occurs as the result of too much cortisol in the blood for an extended period of time. Cortisol is a hormone that, in normal amounts, helps the body perform a number of important functions including converting fat into energy, maintaining immune system function, and responding to stress.

The two types of Cushing's syndrome, exogenous (from an outside source) and endogenous (from a source within the body), share a common list of symptoms but different causes. Exogenous Cushing's syndrome occurs in patients taking cortisol-like medications, and is temporary, ceasing when the patient has finished the course of medication. The endogenous form of this endocrine system disease is far rarer, and results from a tumor or tumors either on the adrenal glands or the pituitary gland.

 

Symptoms

  • Weight gain
  • Muscle loss and weakness
  • Easily-bruised, fragile skin
  • Reduced sex drive
  • Depression/inability to think clearly

 

Treatment

When Cushing's syndrome results from an ACTH-producing tumor of the pituitary gland (Cushing's disease), treatment may include surgery, radiation, or medication to lower cortisol levels.

Surgery — Surgical removal of a small, well-defined pituitary adenoma is called transsphenoidal adenomectomy. The pituitary is located at the base of the brain. It is possible to access this area through the gums above the upper front teeth or the nose.

Using special instruments, the surgeon makes an incision in one of these areas . The incision is extended through the sphenoid sinus, allowing the surgeon to see and remove the adenoma with an endoscope (a thin, lighted tube with a camera). This type of surgery permanently cures Cushing's syndrome in 60 to 70 percent of people.

Sometimes a tumor cannot be identified; in these cases, half of the pituitary gland may be removed (hemihypophysectomy) or 85 to 90 percent of the pituitary gland may be removed (subtotal hypophysectomy) to be certain that the tumor has been removed.

Surgical removal of half or more of the pituitary gland can reduce pituitary function and interfere with ovulation (in women) and sperm production (in men). Lifelong hormone replacement is often necessary after surgery.

Radiation — Radiation can be a useful treatment when pituitary tumors cannot be completely removed by surgery. Radiation of pituitary tumors reduces cortisol levels in about half of adults and most children with Cushing's disease. Because this cortisol-lowering effect takes time (3 to 12 months), medications that lower adrenal cortisol production may be given while waiting for the effects of radiation. These medications include ketoconazole, metyrapone, and aminoglutethimide.

Adrenalectomy — Surgical removal of the adrenal glands (adrenalectomy) is a final measure that may be recommended if other treatments are not successful. Adrenalectomy stops excess cortisol production but requires that you begin lifelong daily glucocorticoid and mineralocorticoid replacement therapy