In a Symposia on Cushing’s Syndrome, chaired by Dr. Beverly M.K. Biller (Mass. General), Dr. James Findling presented on Endogenous Hypercortisolism: Not Your Mentor’s Cushing Syndrome. In his presentation, Dr. Findling emphasized the challenges of diagnosing Cushing’s and discussed the 3 most common screening tests, the 24 hr urinary cortisol, the over-night dex test and night-time salivary cortisol. Dr. Findling pointed out that milder forms of Cushing’s are even more challenging to diagnose and that Cushing’s may be more prevalent than previously thought. This is particularly true in high-risk groups such as diabetics, those with osteoporosis and adrenal incidentalomas.
In the second talk, Dr. Stratakis (NIH) discussed the unraveling of genetic mutations that are being discovered in bilateral adrenal hyperplasias. The third talk by Dr. Bertherat (Inst. Cochin, France) discussed the progress being made in understanding adrenocortical cancer and how this information might become useful for future treatment strategies.
There was also an interesting symposia on signaling pathways in the pituitary where Dr. Drouin (Montreal)discussed possible molecular mechanisms leading to pituitary tumors and the loss of feedback control in ACTH secreting tumors. In this same session, Dr. Heaney (UCLA) discussed potential molecular targets for drug treatment of Cushing’s Disease.
Dr. Bourdeau (Univ of Montreal) presented an oral talk on screening family members of a patient with andrenergic responsive bilateral macronodular adrenal hyperplasia (AIMAH). While none of the family members had overt Cushing’s syndrome, abnormal cortisol responses were found in a substantial number of the patient’s siblings and the following generation. It is becoming increasingly apparent that some cases of AIMAH can be inherited and evaluation of family members can uncover some cases of sub-clinical Cushing’s.
In the poster sessions related to Cushing’s, Dr. Marinoz (Med. Univ. of Sofia, Bulgaria) discussed long term results following transsphenoidal surgery. A number of their patients were followed for an average of 70 months. The cure rate for microadenomas was 87.6% and was 63.6% for larger tumors. Larger tumors and invasive tumors were more likely to recur. At 5 years, the overall recurrence rate was 18.5% and a second surgery for those patients resulted in a 78.6% cure rate.
Dr. Leal-Cerro (Sevilla, Spain) reported on results of memory tests on 14 female patients with active Cushing’s. Over 90% of these patients had memory difficulties and deficits in verbal learning. Dr. Forget (Univ du Quebec en Outaouais) tested 18 patients with Cushing’s for memory and depression issues both prior to cure of Cushing’s and up to 3 years after having normal cortisol levels. Improvements in depression were observed at 12 months and some memory issues were improved at 12 months, while other significant memory improvements were noted at 24 months. Faterme Salehi (Univ of Toronto) developed and validated a questionnaire for quality of life in Cushing’s Disease patients. Further studies will include pre and post treatment results.
Several groups reported on successful use of Cabergoline to normalize cortisol in roughly 1/3 of patients with Cushing’s disease for 1-2 years. In one study (Univ of Montreal) ACTH levels were normalized in 2/8 patients and regression of the pituitary tumor was noted in 1 patient. Unfortunately, Cabergoline carries a risk of cardiac toxicity.
Dr. Barahona (Hosp Sant Pau, Barcelona, Spain) presented a poster titled Coronary Artery Disease in Cushing’s Syndrome Patients Detected by Computed Tomography. In this study of 42 patients, 30 of which were cured, 44% had some evidence of coronary artery disease despite a relatively young age. Risk factors including BMI, BP, length of hypercortisolism, and others were also discussed.
Dr. Ted Friedman (Charles Drew Univ, Los Angeles) reported on the high prevalence of cyclic or episodic Cushing’s in 56 patients with the majority of patients having at least 1 normal test. If only 1 test was performed once, a large number of these patients would not be correctly diagnosed.
There were also several Meet the Professor sessions on Cushing’s given by Dr. Ashley Grossman (St. Bartholomew’s Hosp., London) and Dr. John Newell-Price (Sheffield Univ.UK). Dr. Bertagna (Cochin, France) also gave a session on Nelson’s syndrome.
One of the plenary lectures by Dr. Steven Lamberts (Rotterdam) discussed variations in glucocorticoid sensitivity between individuals and identified several differences (polymorphisms) in the glucocorticoid receptor that might effect this sensitivity. Dr. Lamberts pointed out that such information might be useful in determination of replacement cortisone doses. Another interesting presentation was on the life of Harvey Cushing and his role in the development endocrinology and neurosurgery by Michael Bliss (Univ of Toronto) based on his book, Harvey Cushing: A Life in Surgery (New York, 2005).