Cortisol, a hormone produced by the adrenal glands, helps the body respond to stress. Cushing’s syndrome describes the signs and symptoms of having too much cortisol, such as weight gain, high blood sugar, high blood pressure,and bone thinning. It has many different causes.
Exogenous Cushing’s syndrome occurs when people take too much corticosteroid medication for another condition. Doctors treat this by gradually lowering the dose of corticosteroid to bring down cortisol levels, and substituting nonsteroidal medications to manage the condition.
Endogenous Cushing’s syndrome occurs when the body itself makes too much cortisol. It is most often caused by a tumor in either the pituitary gland or one of the adrenal glands. In either case, Cushing’s disease treatment options include surgery, radiation to destroy tumor cells, or medications to control cortisol levels.
Cushing’s disease refers specifically to Cushing’s syndrome caused by tumors in the pituitary gland. This gland produces many important hormones that regulate body processes. This include adrenocorticotropic hormone (ACTH), which stimulates the adrenal gland to produce cortisol. When a pituitary tumor causes too much ACTH to be produced, the adrenal gland produces too much cortisol.
The primary goal of treating Cushing’s syndrome is to restore normal cortisol balance to reverse the negative effects and prevent complications. The first choice of treatment for Cushing’s disease is to surgically remove the pituitary tumor. Second-choice options include removing the adrenal glands, destroying tumor cells with radiation, and using drugs to lower cortisol levels or block cortisol’s effects.
The first choice of treatment for Cushing’s disease is to surgically remove the ACTH-producing pituitary tumor. The surgeon accesses the pituitary either through the nostril or upper lip, a procedure called a transsphenoidal adenomectomy. The sphenoid sinus is a hollow cavity that sits above the nose and below the pituitary gland.
A neurosurgeon typically performs a transsphenoidal adenomectomy. This successfully reduces cortisol and relieves symptoms in about 70-90 percent of patients, with a higher success rate for smaller tumors. Sometimes tumors return, often because the first surgery left behind some tumor tissue, and a second surgery is needed. Sometimes surgery must be paired with another form of treatment such as radiation therapy or drug therapy.
Some patients need cortisol replacement following surgery, until the body can naturally maintain healthy cortisol levels.
Adrenal gland removal
Another way to bring down cortisol levels is to remove one or two of the adrenal glands, the glands that produce cortisol. Adrenal gland removal is almost always effective, but in almost all cases patients must take cortisol replacement therapy for the rest of their lives. Although too much cortisol causes problems, the body needs cortisol to function properly.
The adrenal glands also make aldosterone, a hormone that controls the balance of salt and water in the body. Patients whose adrenal glands are removed must also take Florinef (fludrocortisone) to replace the functions of aldosterone.
Radiation therapy involves focusing high-energy X-ray beams on the tumor to destroy and shrink it. This is the first treatment for patients who are not candidates for surgery, and as a second treatment when surgery could not completely remove the tumor. It may also be used for larger tumors before surgery, to shrink the tumor to improve chances of successful removal.
Radiation therapy carries a higher risk than surgery of damaging other areas of the pituitary. It also takes longer — up to 10 years — to fully restore cortisol balance and reverse symptoms following radiation therapy. During this time, medications are used to help control cortisol production. Standard radiation therapy to treat pituitary tumors requires multiple treatments over five to six weeks.
Another type of radiation therapy called stereotactic radiation therapy or stereotactic radiosurgery (SRS) targets the pituitary tumor more precisely. It uses a frame to hold the head so the radiation can be directed to the exact location of the tumor. Unlike conventional therapy, stereotactic therapy applies a large dose of radiation in a single treatment, reducing the chances of harming surrounding tissue.
Radiation can destroy other hormone-producing cells in the pituitary, so patients may need replacement therapy with thyroid hormones, growth hormones, sex hormones, or hormones that help control blood pressure (vasopressin).
Drug therapy uses medications to control cortisol levels and is usually combined with surgery or radiation. It may be used as a first treatment when neither surgery or radiation are good options. Drugs used to treat Cushing’s syndrome may be directed at the adrenal glands, the pituitary glands or cortisol receptors. Medical therapy that uses a combination of drugs can improve success because the drugs target different sites to control cortisol balance.
Lysodren (mitotane) and Metopirone (metyrapone) work on the adrenal gland to decrease cortisol levels, Signifor (pasireotide) and cabergoline work on the pituitary to decrease levels of ACTH, and Korlym (mifepristone) blocks the effects of cortisol on body tissues by blocking cortisol receptors. The choice of medication depends on the particular case of each patient. For example, Korlym is most helpful for patients who have diabetes because it is good at controlling blood sugar levels.
Several new experimental drugs are also being explored to treat Cushing’s syndrome.
Treating signs and symptoms
Another aspect of treating Cushing’s syndrome is to prevent the risk of long-term complications due to the effects of too much cortisol on the body. For example, doctors may prescribe medications to treat diabetes, depression or high blood pressure, and use calcium, vitamin D or other medications to prevent weakening of the bones.
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