Long Term Post Operative Follow-Up of Cushing Syndrome

Overview

Although most patients with hypercortisolism can be diagnosed and treated the long-term effects of hypercortisolism and its treatment are unknown. This study will attempt to answer the following questions: 1. What is the rate of perioperative complications? Patients with Cushing syndrome often undergo transsphenoidal surgery of the pituitary gland as treatment for the disease. During this surgical procedure the pituitary gland is reached by passing through the sphenoid bone. The risk for patients to develop hypopituitarism in the immediate postoperative period is unknown. Patients with Cushing syndrome have abnormal levels of hormones circulating in the blood and affects of the surgery are often not apparent until long after the procedure. 2. What is the recurrence rate? The recurrence rate of the disease has been estimated between 5 - 10%. However, these figures have not been confirmed. If the actual rate of recurrence is higher than estimated many patients may elect to undergo radiation therapy which has a lower rate of recurrence. 3. Do any factors in the immediate postoperative period predict who will experience a recurrence of Cushing syndrome? 4. What are the long-term complications of hypercortisolism? Studies have shown that patients with hypercortisolism have a four times greater risk of death than people of the same age without hypercortisolism. Researchers tend to believe this figure is too high. However, it is well established that hypercortisolism weakens bones (decreased bone density), causes secondary hypogonadism, increases levels of fat in the blood (hyperlipidemia), and decreases thyroid function (hypothyroidism). The potential for these conditions to be reversed is not known. These questions will be addressed by blood and urine sampling in the postoperative period, and by outpatient follow-up and periodic questionnaires in the first 10 years after curative surgery for Cushing syndrome performed at the NIH.

Although most patients with hypercortisolism now can be diagnosed correctly and successfully treated, the long-term sequelae of hypercortisolism and its treatment are unknown. This study addresses the following questions: 1) What is the recurrence rate after successful treatment of Cushing's syndrome? 2) Do any factors in the immediate postoperative period predict recurrence of Cushing's syndrome? 3) What are the long-term complications of hypercortisolism in terms of mortality, morbidity, return of endocrine function and bone density? and 4) What is the quality of life for patients after surgical treatment of Cushing's syndrome? While most of these questions relate specifically to the care of patients with Cushing syndrome, the final question has relevance to the many patients rendered Cushingoid by the therapeutic use of glucocorticoids. These questions will be addressed by blood and urine sampling in the post-operative period, and by outpatient follow-up and periodic questionnaires in the first 20 years after curative surgery for Cushing syndrome performed at the NIH.