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Will BLA Solve My Problem?

Question:  I have confirmed hypercortisolism (enough highs after several months of testing) but no clear target.  I’m morbidly obese, have lost my period, have new hair growth on my face, and almost all of the other classic Cushing’s symptoms.  If I have a bilateral adrenalectomy and just remove the source of cortisol production, will these symptoms go away?  I’m willing to have adrenal insufficiency if it means these other things stop.

Answer 1:  They probably would, but BLA should be reserved for cases in which no other option is available. You need a diagnosis (do you really have Cushing, and if so, what is the cause? Is it ACTH dependent or independent?). Therapy will be then targeted to the underlying problem – pituitary adenoma, adrenal tumor, etc.  (Dr. Roberto Salvatori, Johns Hopkins Pituitary Center)

Answer 2:  If ACTH-dependent hypercortisolism is confirmed and nothing is on the brain MRI, an IPSS can help locate a minuscule pituitary adenoma or if the source is outside of the pituitary (ectopic).  If ectopic source is confirmed you could have an octreoscan or even better a dotatate scan to try and find it and remove it.  I would first try to find the culprit tumor before taking out the adrenals.  If all the body changes are related to the excess of cortisol you should have significant improvement but it is important to have an in-depth discussion with your doctor so you have realistic expectations regarding the recovery.  (Dr. Georgiana Dobri, Cornell)

Answer 3:  It is very important to answer this question in detail.  Due to many false positives encountered in hypercortisolemia testing, Cushing’s should be clearly confirmed – by elevation of cortisol (24 UFC and late night salivary cortisol), non-suppressibility to dexamethasone, and careful localization work-up.  If complicated, testing preferably to be done in a tertiary Center.

No treatment should be started without clear diagnosis of Cushing’s and establishing exact etiology, as there are many risks associated with each type of treatment. Recovery post- treatment depends on many factors, including severity and duration of the disease, but typically symptoms and features related to hypercortisolemia will slowly improve with time.  (Dr. Maria Fleseriu, Oregon Health and Science University)

Answer 4:  This is a very important issue, especially with the Internet. We are a referral center for pituitary disorders and Cushing’s patients, so I see a real spectrum – patients who have Cushing’s and patients who suspect it is the cause of their weight issues, diabetes, depression, etc.

I explain that all of our screening tests are equally reliable: approximately 92% reliable [late night salivary cortisol, 24 hour urine free cortisol and 1 mg dexamethasone test]. I offer to re-test patients with multiple tests.

Because of the overlap of signs and symptoms with simple obesity and polycystic ovary syndrome, it may be difficult to assess these women clinically, so one thing I’ve learned over the years (and from the late Dr. Edward Oldfield) – sometimes “time” is the best test – so if there is a question and variable test results, I will see the patient again in 4-6 months.  (Dr. Mary Lee Vance, University of Virginia)

 Answer 5:  Bilateral adrenalectomy is usually the last resort when other therapies have failed.  Unfortunately despite progress in surgical and radiotherapy techniques several patients remain with active CD. Fortunately novel medical therapies now provide interesting options to control disease, but most of them are imperfect to restore completely normal HPA axis physiology or have side effects with impact on quality of life. Some issues result from financial costs with some medical therapies particularly when young patients consider chronic therapy over decades. Some young woman with residual CD desire fertility and cannot combine this with most medical therapies when cabergoline is not effective. So there are still some indications for bilateral adrenalectomy, but this has to be evaluated carefully in a multidisciplinary team approach with a well-informed patient in referral center with wide experience in CD and capacity for long term follow-up of permanent adrenal insufficiency, teaching in its care and continued follow-up of residual corticotroph tumor and co-morbidities. (Dr. André Lacroix, Université de Montréal)

 Editor’s Note: Dr. Lacroix included a research article entitled “Bilateral Adrenalectomy in the 21st Century: When to Use it for Hypercortisolism?” with his answer – click here to read it.

(Winter-Spring 2019)

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