Saturday, February 14, 2015

Another Phaeo

Phrase that sends shivers up an endocrinologist's spine:
"Biopsy-proven phaeochromocytoma..."
Saw my 2nd case of this recently, to my chagrin.
I've seen perhaps 20 cases in my career so far of catecholamine secreting neuroendocrine tumors. But theses should be diagnosed based on clinical suspicion, appropriate biochemistry and radiologic findings.
Never (usually- but will come back to it*) is it justified to diagnose this from a biopsy, as this usually means the physician/surgeon evaluating a patient with an adrenal mass was not considering a phaeo in the differential diagnosis. Or that he/she made the fatal assumption that asymptomatic rules out a phaeo and that lab testing isn't needed- just stick a needle into it.
My first referral was 7 years ago- that patient had a hypertensive crisis in the radiologic suite immediately following the adrenal biopsy. I think the radiologist shat in his scrubs when it happened and it must immediately have dawned on him what the mass actually was.
The second, this patient, had adrenal hemorrhage that required invasive intervention.
What I tell residents is this: The classic features of a phaeo isn't that classic after all. All adrenal masses need a biochemical workup, especially before you stick a needle into it.
Most phaeos are asymptomatic. And of the symptomatic ones, the most common is hypertension; what looks to be plain old simple essential hypertension. So never assume.
(*perhaps the one situation in which I would not blame this on shoddy work is a paraganglioma/extraadrenal phaeo in a weird location´╗┐- which would be unfair to expect one to consider a phaeo in the differential diagnosis)