Treating Cushing syndrome

Transsphenoidal surgery (TSS)

Overview

TSS is a surgical procedure to remove a nodule on the pituitary gland, located at the base of the brain.

The surgery may be guided by a camera inserted through a small tube (endoscopy) or by using a specialized microscope designed to magnify the surgical site.

TSS is recommended as the first choice for treatment in people diagnosed with Cushing disease.

Things to consider

• With TSS, the function of the pituitary gland may be preserved. This allows the body to continue producing hormones on its own without medication

• Many people treated with TSS go into remission from their Cushing syndrome symptoms

• In some people, TSS does not resolve Cushing syndrome

• Patients who go into remission may still have recurrences (symptoms that come back) and require further treatment

Adrenalectomy

Overview

An adrenalectomy is a surgical procedure to remove one or both adrenal glands, located above the kidneys.

Typically, the surgery is performed through laparoscopy (a type of minimally invasive abdominal surgery). The surgery is guided by a camera inside a thin tube inserted through a small incision in the abdomen.

Removal of both adrenal glands (bilateral adrenalectomy) is recommended only in patients with Cushing disease who have had treatment failure with TSS and radiotherapy or who are not candidates for TSS or radiotherapy.

In cases with an adrenal source, the affected adrenal gland may be removed (unilateral adrenalectomy). If both adrenal glands are affected, both glands may be removed, also known as a bilateral adrenalectomy.

Things to consider

• Remission of Cushing syndrome symptoms may be achieved by removing the adrenal glands

• This procedure is irreversible

• People who have a bilateral adrenalectomy require lifelong medication to replace the cortisol their bodies can no longer produce

Radiotherapy

Overview

Radiotherapy is a general term that covers several different treatment techniques.

Radiation may be delivered using a precisely targeted method called stereotactic radiosurgery (this includes the “Gamma Knife” method, which uses a specialized device to administer targeted radiation to a particular site within the body). Conventional radiotherapy uses lower doses spread over multiple sessions and may also be used for people with Cushing syndrome.

Radiation may be administered on a single occasion or during multiple sessions.

Response to radiotherapy is evaluated by measuring cortisol levels. Cortisol levels should be periodically monitored to look for recurrences.

Radiotherapy is recommended for people diagnosed with Cushing disease who:

• Are not candidates for TSS

• Were unsuccessful with TSS

• Experienced a recurrence of symptoms after TSS

Things to consider

• Radiotherapy is effective for treating the growths that cause Cushing disease and may help prevent these growths from coming back

• Treatment may take some time to begin working. It is not uncommon for people to continue experiencing symptoms for a long time before seeing results from radiotherapy

• There is some risk of the pituitary becoming inactive and no longer releasing necessary hormones

Medical therapy

Overview

Medication may play an important role in the treatment of Cushing syndrome. Sometimes medication may be used before surgery to help control symptoms or to confirm whether Cushing syndrome is the underlying cause of symptoms. Medication may also be used if a person is not eligible for surgery, has failed surgery, or has experienced a recurrence following surgery. For patients receiving radiotherapy, medication is sometimes used supportively as the effects of radiotherapy are not immediate.

Some ways that different medications may work are:

• Blocking the adrenal glands from producing cortisol

• Blocking or controlling hormones that signal the adrenal glands to produce cortisol

• Competing with cortisol at glucocorticoid receptors throughout the body to reduce cortisol activity

Things to consider

Because medical therapies work differently to control cortisol in the body, there may be additional considerations for specific medical treatments. When considering medical therapy, you may think about the following:

• Medication is noninvasive and may be stopped if not tolerated

• Medication must be taken for the rest of your life to be effective (unless surgery or radiotherapy are performed successfully)

• Some people may experience negative side effects from treatment. Frequent monitoring may be required