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Cushing's Syndrome & Disease - Treatments

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What are the Treatment Options for Cushing’s Disease?

The only effective treatments for Cushing’s disease are to remove the tumor, to reduce its ability to make ACTH, or to remove the adrenal glands. There are other complementary approaches that may be used to treat some of the symptoms. For example, diabetes, depression and high blood pressure will be treated with the usual medicines used for these conditions. Also, doctors may prescribe additional calcium or vitamin D, or other medicine to prevent thinning of the bone.

Pituitary Tumor Removal by Surgery
Removal of the pituitary tumor by surgery is the best way to treat Cushing’s disease. This is recommended for those who have a tumor that is not extending into areas outside of the pituitary gland, and who are well enough to have anesthesia. This is usually carried out by going via the nose or upper lip and through the sphenoid sinus to reach the tumor. This is known as transsphenoidal surgery and avoids having to get to the pituitary via the upper skull. This route is less traumatic for the patient and allows quicker recovery.

Removing only the tumor leaves the rest of the pituitary gland intact so that it will eventually function normally. This is successful for 70–90% of people when performed by the best pituitary surgeons. The success rates reflect the experience of the surgeon performing the operation. However, the tumor can return in up to 15% of patients, probably because of incomplete tumor removal at the earlier surgery.

Radiosurgery
Other options for treatment include radiation therapy to the entire pituitary gland or targeted radiation therapy (called radiosurgery), when the tumor is seen on MRI. This may be used as the only treatment or it may be given if pituitary surgery is not completely successful. These approaches can take up to 10 years to have full effect. In the meantime patients take medicine to reduce adrenal gland production of cortisol. One important side effect of radiation therapy is that it can affect other pituitary cells that make other hormones. As a result, up to 50% of patients need to take other hormone replacement within 10 years of the treatment.

Adrenal Gland Removal by Surgery
Removal of both adrenal glands also removes the ability of the body to produce cortisol. Since adrenal hormones are necessary for life, patients must then take a cortisol-like hormone and the hormone florinef, which controls salt and water balance, every day for the rest of their life. An experienced pituitary- or neuro-endocrinologist can help to decide the best course of treatment.

Drug Treatments
While some promising drugs are being tested in clinical studies, currently available medications to reduce cortisol levels, when given alone, do not work well as a long-term treatment. These medicines are most often used in conjunction with radiation therapy.

Transsphenoidal Surgical Approach

Use of Magnetic Resonance Imaging (MRI) to Visualize the Pituitary Gland