These pituitary tumors make a hormone called adrenocorticotropic hormone (ACTH), which stimulates the adrenal glands to make proteins called glucocorticoids. When the body makes too much ACTH, it causes a disorder known as Cushing's disease. Most patients who develop this disorder are women in their 30s and 40s. ACTH-producing tumors account for 10 to 15 percent of pituitary tumors.

Symptoms

Patients with Cushing's disease may experience unexplained weight gain, diabetes, increased blood pressure, easy bruising, moodiness or depression, and irregular or absent menstrual periods.

Diagnosis

Cushing's disease must be distinguished from several other diseases that can increase the production of hormones from the adrenal glands. Cushing's disease is diagnosed using blood tests that look for abnormal secretion of a hormone called cortisol. MRI may also be employed, but sometimes a pituitary tumor that causes Cushing's disease is so small that it may not be visible using this imaging test. The diagnosis, therefore, must often be confirmed during surgery, when the surgeon removes the tumor and has it biopsied.

Treatment

Surgery to remove the tumor is the primary therapy for Cushing's disease. Surgery for pituitary tumors is often performed through a minimally invasive approach called transphenoidal hypophysectomy, whereby the surgeon removes the tumor through an incision in the nasal passage. In cases where the tumor is too large to be removed through this approach, the surgeon performs a craniotomy, removing the tumor through an incision in the front of the skull.

If surgery alone is not curative, patients may take a drug called ketoconazole (Nizoral). This drug is most often used to treat fungal infections, but when given in high doses, it shuts down production of ACTH by the adrenal glands. In rare cases, when even this approach is not effective, the adrenal glands may be surgically removed.

Follow-Up

Your doctor will see you periodically and perform certain tests to ensure that your pituitary tumor has not returned. If medication is part of your treatment, you may need to take it for the rest of your life to prevent tumor recurrence.