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40 Cards in this Set

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What regulates production/secretion of aldosterone?
Angiotensin II
Serum potassium
(very little by ACTH)
What is main inhibitor of aldosterone secretion?
ANP
What is Cushing syndrome?
Hypercortisolism secondary to excessive production of glucocorticoids by the adrenal cortex
What organ and/or substances stimulate secretion of ACTH, and from what organ is it secreted?
Hypothalmus acts on the ant pituitary via CRH, oxytocin, and vasopressin
What substance inhibits ACTH secretion?
Cortisol, acting on the hypothalamus and ant pituitary
When is the highest level of cortisol detected in humans?
Morning
(nadir is at 11pm)
When is bilateral adrenalectomy indicated for Cushing syndrome (3)?
1. For Cushing disease: when at least one attempt to treat the primary tumor has failed
2. For ectopic ACTH syndrome: if primary tumor is unresectable
3. Rarely - when hypercortisolism is life threatening and swift definitive reduction is necessary
What is Nelson syndrome?
Progressive growth of pituitary adenoma after bilateral adrenalectomy, which results in expected neurological issues d/t mass effect (visual disturbances, etc)
What two things might a pt with undiagnosed Cushing syndrome present to urologist with?
1. Hypogonadal hypogonadism
2. Nephrolithiasis
The serum level of what substance distinguishes primary from secondary aldosteronism?
Renin
(low in primary; elevated in secondary)
What 2 entities account for 95% of the cases of hyperaldosteronism?
Benign adrenal hyperplasia (60%)
Aldosterone producing adenomas (35%)
[of note, primary aldosteronism associated w/ adrenal cortical carcinoma is rare]
Which patients w/ primary aldosteronism should get an abdominal CT?
Any that is a surgical candidate (to look for resectable adenomas)
What percentage of incidental adrenal masses are pheochromocytomas?
5%
What percentage of pheochromocytomas are familial?
30%
What familial syndrome associated with pheochromocytomas is most relevant to urology? Why?
VHL (type 2)
Associate w/ RCC
What defines a pheochromocytoma as malignant?
Metastatic disease
Malignancy is more common is which two subgroups of pheochromocytomas?
Extra-adrenal (33%)
SDHB mutation (30-50%)
What are the CT and MRI characteristics of pheochromocytomas?
CT: >10 HU (mean ~35 HU)
MRI: High intensity on T2
What biochemical test is most sensitive for detection (screening) of pheochromocytomas?
Metanephrines
a. Plasma-free metanephrines
b. 24-hr fractionated urinary metanephrines
What medication should be given pre-operatively prior to resection of a pheochromocytoma? What other additional meds may be used if necessary?
Phenoxybenzamine (irreversible alpha blocker)

Beta blockers (atenolol, metoprolol)
Metyrosine (catecholamine synthesis blocker)
CCBs
What percentage of adrenocortical carcinomas are functional?
50-80%
What is the most common hormone secreted by ACCs?
Cortisol (causing Cushing syndrome)
What is the average size of ACCs on CT at presentation?
10 to 12 cm
(over 90% > 5 cm)
Adrenal tumors greater than what size should be resected?
4 to 6 cm
What is the metastatic workup for ACC?
CT of chest/abd/pelvis
(Brain and bone imaging only if symptomatic)
What are typical HU for ACCs on CT?

What are other common CT characteristics of ACCs (4)?
39 HU on non-contrast CT (compared to 8 HU for adenomas)

1. Irregular borders
2. Irregular enhancement
3. Calcifications
4. Necrotic areas w/ cystic degeneration
How is "washout phase" imaging on CT used to diagnose adrenal lesions?
Both lipid rich (<10 HU) and lipid poor (>10 HU) adenomas will have prompt washout of contrast on delayed CT images
(Non-adenoma lesions will not demonstrate washout)
When should an adrenal mass be biopsied?
Pretty much never
(All imaging and biochemical tests have been exhausted, suspicion of ACC is low, and results will clearly affect clinical decision making)
What imaging study is the gold standard for evaluation of adrenal incidentalomas?
CT Washout Study
What are the criteria for diagnosing an adrenal adenoma on a CT washout study?
Absolute percent washout >60%

OR

Relative percent washout >40%
(use when non-contrast images aren't available)
What change in size on follow-up imaging is considered criteria for adrenalectomy?
>1 cm growth
All new adrenal masses should be tested for hypersecretion of what?
Cortisol and catecholamines
(aldosterone if hypertensive)
What 3 tests can be used to screen for cortisol hypersecretion?
1. Overnight low-dose desamethasone suppression test
2. Late night salivary cortisol test
3. 24 hour urinary free cortisol evaluation (not recommended by some due to decreased sensitivity)
What common medication can yield false positive results on low dose dexamethasone suppression test?
OCPs
What common substance can affect the results of the salivary cortisol test?
Tobacco (avoid on day of test)
What test should be used to screen for hyperaldosteronism?
Morning plasma aldosterone to renin ration (ARR)

[ARR = 20 (or 30) w/ aldosterone >15 ng/ml is diagnostic]
What medications should be stopped before screening for hyperaldosteronism and when?
Potassium sparing diuretics, especially spironolactone (also amiloride)

Stop 6 weeks before testing
What common antihypertensive agents do not need to be discontinued prior to screening for hyperaldosteronism?
CCB, ACE inhibitors, and ARBs
(beta blockers can cause false positive results; K-sparing diuretics must be stopped)
Is routine testing of adrenal incidentalomas for sex hormones currently recommended?
No
What medications can produce false positive results on plasma free metanephrine testing?
Acetaminophen (assay cross-reactivity)
TCAs
Phenoxybenzamine