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  #1  
Old 10-18-2011
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Question Elevated VMA, what's the best next step?

A 35-year-old woman has been complaining of severe headaches, with palpitations and sweating, which occur at random times during the day and last from 10 to 20 minutes. By coincidence, she has one of these episodes while waiting to be seen by her physician, and the office nurse determines that her blood pressure at the time was 220/140 mm Hg. Subsequent testing shows elevated levels of vanillylmandelic acid, norepinephrine and normetanephrine in a 24-hour urinary collection. In that same test, levels of epinephrine and metanephrine are normal. CT scan of her adrenal glands is likewise unremarkable. Which of the following is the most appropriate next step in management?

A. Alpha blockade and exploratory laparotomy
B. Beta blockade and bilateral adrenal exploration
C. Iodocholesterol scintigraphy (59 NP scan) of the chest and abdomen
D. MRI of the adrenal glands
E. Whole-body radioactively labeled I-metaiodobenzylguanidine (MIBG) scan
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Old 10-19-2011
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I will go with E - only because other choices dont really make sense to me and I like the way the test sounds. I'm also thinking there is a neural tumor that may be located outside the adrenals.

Whats the answer?
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Old 10-19-2011
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E. Whole-body radioactively labeled I-metaiodobenzylguanidine (MIBG) scan
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Old 10-19-2011
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The correct answer is E. The clinical diagnosis is pheochromocytoma, but all indications are that it is located at an extraadrenal site: the tumor makes only norepinephrine, and it is not visible on CT of the adrenals. Pheochromocytomas are typically quite large by the time they are symptomatic, and thus the negative CT scan is significant. This tumor could be located anywhere from the neck to the pelvis, and thus a whole-body scan is indicated with a substance (MIBG) known to be taken by chromaffin tissue in direct proportion to its biosynthesis of catecholamines. MRI can then be directed to the appropriate area for better visualization of the tumor.
Exploring the adrenal glands, either through the abdomen (choice A) or through the flank (choice B), would be inappropriate when all evidence points to an extraadrenal tumor site. Additionally, choice B is flawed because it suggests operating on a patient with a pheochromocytoma with only beta blockade can lead to severe, even lethal, hypertensive crisis as a result of unopposed alpha-adrenergic agonism.
The 59NP scan (choice C) is used to identify aldosterone-producing adenomas.
An MRI (choice D) is a better test than CT to distinguish pheochromocytoma from benign adrenocortical adenomas, but MRI will not show a tumor that is not there on the CT.
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