QUESTION--hello and thank you very much for all efforts you are doing to promote endocrinology. , i am a fellow in training , in my last year (4th) endocrinology and métabolisme -constantine university-algeria and i am writing to you for the second time. i have a case wich create us a lot of challenge.
CH A patient aged 33,female. No previous history of any pathology
she has a problem on the management of recurrent Cushing syndrome..In 2008, an unencrypted obesity with some stretch marks, but unexplored .
In 2009, a sudden onset of HIC syndrome with headache, vomiting, and decreased visual acuity,The patient put under mannitol and high dose corticosteroids for 5 days.
A brain MRI found a necrotic macro-pituitary adenoma necrotic.
The biological exploration revealed a normal hypophysiogramme, except for a slight increase in TSH 6mui / ml (0.4-4), and low cortisol few days after stopping methylprednisolone down on several occasions. The patient is put with the diagnosis of pituitary adenoma apoplexy complicated by adrénal failure and the patient was put under hydrocortisone 30mg / d.
From 2009 to 2012 the course was marked by regression of obesity .the disappearance of headache and regression of stretch marks, the patient remained under hydrocortisone.
In January 2012, with the reappearance of obesity, an assessment made under treatment found high TSH 10 mIU / ml (0.4-4), the rest of the pituitary hormons was normal and an MRI done by the patient found a partially empty sella. The endocrinologist stopped hydrocortisone and gave Levothyroxine 50microgramme / day .
In late 2012, the patient was hospitalized for a cardiac emergency acute coronary syndrome , put under aspirin high dose and clopidogrel, coronary angiography found healthy coronary arteries,and levothyroxine was arrested
In 2014 and with the exaggeration of obesity with faciotronculaire distribution and occurrence of hirsutism, purple stretch marks and psychiatric disorders like depressive tendencies, an endocrine exploration revealed a very high urinary free cortisol 400 microgram / 24h twice with ACTH 70pg / ml (high), the rest of the hypophysiogramme was normal, and the diagnosis of ACTH-dependent Cushing syndrome was selectedThere are a few months the patient consulted us, exploration done is as followings:
High urinary free cortisol ( twice)
Rest of hypophysiogramme is normal
Low dose dexamethasone suppression test : cortisol after suppression :5microgramme/dl
high dose dexamethasone suppression test : ongoing
MRI: partially empty sella and no detectable adenoma
Catheterization of inferior petrosal sinus is not available in Algeria
CT abdomen and thorax: Normal
Brochoalviolaire washing; not objectified malignancies
What would you recommend?
1 / Retain the diagnosis of a recurrence of Cushing's disease?
2 / If so what do you recommand as a treatement ?
a / Make an exploratory surgery
B / Provide medical treatment
c / or give radiotherapy
d / Or repeat MRI periodically pending the appearance of adrenocorticotropic adenoma
Thank you. nadjib kaouache, MD [mailto:firstname.lastname@example.org
RESPONSE- Thanks, this does sound like a case of a pituitary macroadenoma causing Cushing;'s disease which underwent partial infarction and has now recurred. However, the tumour is not clearly visible and is unlikely to become so for some time. In the absence of the ability to perform petrosal sinus sampling, but no evidence of a (second) ectopic source, I think we can assume that there is a small occult corticotroph tumour within the pituitary. Surgery is unlikely to find this and will have a high risk of a CSF leak.
My feeling would be that you should control the hypercortisolaemia with ketoconazole +/- metyrapone, and then consider bilateral adrenalectomy. Thereafter, periodic scanning of the pituitary should be performed, with measurements of ACTH a couple of times a year.Best wishes, Ashley Grossman,MD (And check possible hypothyroidism)