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.
Patients with Cushing syndrome documented at the NIH will be recruited into this protocol
prior to surgery intended to cure Cushing syndrome selectively. Thus, patients undergoing
transsphenoidal exploration for resection of an adenoma, or unilateral adrenalectomy for
an adenoma, or resection of a tumor producing ACTH ectopically will be candidates for this
study. We will also recruit as many of the 500 patients treated since 1983 as possible,
so as to have a group of "late follow-up" patients. These individuals will participate
primarily by questionnaire.
Hematocrit about 30%. A CBC will be obtained prior to entering the study. Patients with
hematocrit above 30% will be accepted into the study; iron replacement will be given to
those patients with low TIBC.
Ages 18 - 85. Children less than 18 are being studied under other protocols and the
questionnaire has not been validated for younger individuals.
For the questionnaire portion of the protocol there is an additional inclusion criterion:
Patients must be able to read and write in English.
There are no formal exclusion criteria, if the patient meets the inclusion criteria above.
All ethnic groups and both genders will be recruited. However, patients may be
withdrawn from the study by the PI if they are unable to meet study requirments, such as