Sunday, May 25, 2008

Cushing's- A Challenge


Am oncall this week, and next, and among the cases I've seen is a really challenging case of Cushing's.

This was a young man, nice person, good-looking even (could have been a model, so his mother says). And then suddenly, he began putting on weight at an alarming rate, developed weird stretch marks and his muscles wasted away. Basically all the features of Cushing's syndrome (a condition of excess steroids).

As it turns out, he was found to have metastatic neuroendocrine tumor producing ACTH. Hence the rapidly progressive course (which is a clue to the etiology of Cushing's- pituitary versus ectopic).

Underwent bilateral adrenalectomy, and despite the very skilled surgeon, the surgery took well over 10 hours and the surgeon was not able to remove the right gland entirely, which was basically stuck to the liver from the mets.

So now, he's still on the ventilator, and we're trying to wean him off. His transaminases are sky-high, which will limit what else we can do for his Cushing's which is presumed to be still present.
Medical therapy with Ketoconazole? Nope. Mitotane? Nope. Etomidate? Nope. Metyrapone? Well, maybe, if we can get our hands on it.
A consultant suggested ethanol ablation of the remnant adrenal- not sure if this is feasible.

So, what we're left with is wait and pray, and hope that he's extubated and his liver recovers.

And the worse thing is (and this is my own biased emotions, I admit) he's only my age, with a young family. I suppose that's human nature, unreasonable as it may seem, that sickness and death are easier to accept in the elderly, not in the young.