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Fiser's ABSITE Ryan SHORT > Chapter 21 - Adrenal > Flashcards

Chapter 21 - Adrenal Flashcards

1
Q

Arterial supply of the adrenal?

A

Superior adrenal (from inferior phrenic), middle adrenal (from aorta), inferior adrenal (from renal artery)

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2
Q

Venous drainage of adrenal?

A

Left adrenal to L. renal vein, R. adrenal to IVC

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3
Q

What % of CT scans show incidentaloma?

A

1-2%

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4
Q

When is surgery indicated for incidentaloma?

A

Ominous characteristics (nonhomogenous), >4-6cm, functioning, enlarging

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5
Q

How often do you need to follow an incidentaloma?

A

Q3 months for 1 year, then yearly

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6
Q

How do you work up an incidentaloma?

A

Serum K, urine metanephrines/VMA/catecholamines, urinary hydroxycorticosteroids, plasma renin and aldosterone levels if HTN or dec. K; CXR, stool guiac and colonoscopy, mammogram

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7
Q

What are common mets to the adrenal?

A

Lung CA (#1), breast, melanoma, renal

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8
Q

What do you do for a patient with cancer history and an asymptomatic adrenal mass?

A

Biopsy

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9
Q

Adrenal cortex layers and products?

A

GFR = salt, sugar, steroids; glomerulosa (aldosterone), fasciculata (glucocorticoids), reticularis (androgens/estrogen)

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10
Q

Adrenal medulla receives innervation from what?

A

Splanchnics

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11
Q

What causes the release of cortisol from the adrenal cortex?

A

CRH (hypothalamus) - ACTH (anterior pituitary) - release of cortisol

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12
Q

When does cortisol peak?

A

Diurnal, 4-6am

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13
Q

What are the effects of aldosterone?

A

Stimulates renal sodium resorption and secretion of K+, H+, and ammonia

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14
Q

What stimulates secretion of aldosterone?

A

Angiotensin II, hyperkalemia, ACTH

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15
Q

What 3 deficiencies cause congenital adrenal hyperplasia?

A

21-hydroxylase deficiency(90%), 11-hydroxylase deficiency, 17-hydroxylase deficiency

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16
Q

Characteristics of 21-hydroxylase deficiency?

A

Precocious puberty in males, virilization in females; inc. 17-OH progesterone leads to inc. production of testosterone; salt wasting causing hypotension

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17
Q

Treatment for congenital adrenal hyperplasia?

A

Cortisol, genitoplasty

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18
Q

Characteristics of 11-hydroxylase deficiency?

A

Precocious puberty in males, virilization in females; salt saving causing hypertension

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19
Q

Characteristics of 17-hydroxylase deficiency?

A

ambiguous genitalia in males at birth; salt saving

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20
Q

Symptoms of hyperaldosteronism (Conn’s syndrome)?

A

HTN without edema, hypokalmia, weakness, polydipsia, polyuria

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21
Q

Causes of primary hyperaldosteronism (low renin)?

A

Adenoma (80-90%), hyperplasia, ovarian tumor, cancer

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22
Q

Causes of secondary hyperaldosteronism (high renin)?

A

CHF, renal artery stenosis, liver failure, pregnancy, diuretics, Bratter’s syndrome

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23
Q

What is Bratter’s syndrome?

A

Renin-secreting tumor

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24
Q

How is the diagnosis of primary hyperaldosteronism made?

A

Urine aldosterone after salt load (will stay high); low serum K, high urine K, high serum Na, metabolic alkalosis; aldosterone:renin ratio >20

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25
What is the NP-59 scintigraphy?
Shows hyper functioning adrenal tissue, differentiates adenoma from hyperplasia
26
Treatment for adenoma causing hyperaldosteronism?
Adrenalectomy
27
Treatment for hyperplasia causing hyperaldosteronism?
Medical tx 1st with spironalactone, ca-channel blocker, potassium
28
What is the #1 cause of hypocortisolism (Addison's disease)?
Withdrawal of exogenous steroids
29
What is the #1 priamry disease causing hypocortisolism?
Autoimmune disease
30
Hormone/electrolyte aberrations with hypocortisolism?
Low cortisol and aldosterone; low serum Na, high serum K
31
Symptoms of acute adrenal insufficiency?
Hypotension, fever, lethargy, abdominal pian, low glucose, depressed mental status, nausea/vomiting, high K+
32
Treatment of adrenal insufficiency?
Dexamethasone, fluids, ACTH stim test
33
Most common cause of hypercortisolism (Cushing's syndrome)?
Iatrogenic
34
How is the diagnosis of hypercortisolism made?
1st: 24h urine cortisol (most sensitive), 2nd: low dose overnight dexamethason suppression test (test urine to see if the cortisol has been suppressed - if low, Cushing's disease, if high, go to 3rd), 3rd: serum ACTH (if high, ectopic ACTH or pituitary tumor and go to 4th, if low ACTH, pt has cortisol-secreting tumor), 4th: serum ACTH high - high dose overnight dexamethasone suppression test (positive = pituitary, negative = ectopic), 5th: CRH test - pituitary adenomas will increase ACTH, ectopic producers will have no change in ACTH; MRI useful, NP-59 to localize and differentiate from hyperplasia
35
#1 noniatrogenic cause of Cushing's syndrome?
Pituitary adenoma (70-80%)
36
Test findings with pituitary adenoma?
Cortisol should be suppressed with either low- or high-dose dexamethasone suppression test
37
Treatment of pituitary adenoma?
Transsphenoidal resection; unresectable or residual tumors treated with XRT
38
#2 noniatrogenic cause of Cushing's syndrome?
Ectopic ACTH - most commonly from small cell lung cancer
39
Test findings with ectopic ACTH?
Cortisol is NOT suppressed with either low-or high-dose dexamethasone suppression test
40
Treatment of ectopic ACTH secreting tumor?
Resection of primary if possible; medical suppression or bilateral adrenalectomy for inoperable lesions
41
#3 noniatrogenic cause of Cushing's syndrome?
Adrenal adenoma
42
Test findings with adrenal adenoma causing Cushing's?
Low ACTH, unregulated steroid production; does not suppress
43
Treatment for adrenal hyperplasia?
Bilateral adrenalectomy
44
Medical therapy for ectopic ACTH production or adrenocortical cancer with residual or metastatic disease after resection?
Ketoconazole/metyrapone (inhibit steroid fomation), aminoglutethimide (inhibits cholesterol conversion), Op-DDD (adrenal-lytic, used for metastatic disease)
45
Postoperative care of bilateral adrenalectomies includes what?
Steroids
46
Age distribution of adrenocortical carcinoma?
Bimodal (before age 5, in the 5th decade)
47
% of adrenocortical carcinomas that are functional?
50% cortisol, aldosterone, sex steroids
48
Symptoms seen in children with adrenocortical carcinoma?
90% with virilization (precocious puberty in boys, virilization in females)
49
Symptoms of adrenocortical carcinoma?
Abdominal pain, weight loss, weakness
50
Treatment of adrenocortical carcinoma?
Radical adrenalectomy; mitotane for residual or recurrent disease
51
Survival rate of adrenocortical carcinoma?
20% 5-year survival
52
What is the adrenal medulla derived from?
Ectoderm neural crest cells
53
Steps of catecholamine production?
Tyrosine --> dopa --> dopamine --> norepinephrine --> epinephrine
54
What is the rate-limiting step in catecholamine production?
Tyrosine hydroxylase (tyrosine to dopa)
55
What enzyme converts norepi to epi (requires methylation)?
PNMT, found only in the adrenal medulla
56
What is the only tumor that will produce epinephrine?
Adrenal pheochromocytomas
57
What enzyme converts noepi to normetanephrine and epi to metanephrine?
Monoamine oxidase (MAO)
58
Where is extra0adrenal neural crest tissue found?
In the retroperitoneum, organ of Zuckerkandl
59
What do pheochromocytomas arise from?
Chromaffin cells; arise from sympathetic ganglia or ectopic neural crest cells
60
What is the 10% rule with pheos?
10% malignant, bilateral, in children, familial, extra-adrenal
61
What syndromes are pheos associated with?
MEN IIa, MEN IIb, von Recklinghausen's disease, tuberous sclerosis, Sturge-Weber disease
62
Which side has more pheos?
Right side
63
Are extra-adrenal tumors more likely malignant or benign?
Malignant
64
Symptoms of pheos?
HTN (episodic), headache, diaphoresis, palpitations
65
How is the diagnosis of pheo made?
Urine mtanephrines and VMA (VMA most sensitive), MIBG scan (norepi analogue) to localize, clonidine suppression test (tumor does not respond, keeps catecholamines high)
66
Is venography used for pheos?
NO, can cause hypertensive crisis
67
Preoperative treatment of pheos?
Volume replacement, alpha-blockers (phenoxybenzamine), then beta-blocker if pt has tachycardia or arrhythmias
68
What causes hypertensive crisis with pheos?
Unopposed alpha stimulation; can be precipitated by beta blockers given before alpha blockers; can cause heart failure in patients with cardiomyopathy
69
Treatment of pheo?
Resection, check for other tumors, ligate adrenal veins to avoid spilling catecholamines; debulking if unresectable; metyrosine (inhibits tyrosine hydroxylase)
70
What are frequent post op conditions following pheo resection?
Persistent hypertension, hypotension, hypoglycemia, bronchospasm, arrhythmias, intracerebral hemorrhage, CHF, MI
71
What are extra-adrenal sites of pheos?
Vertebral bodies, bladder, aortic bifurcation
72
Where is the organ of Zuckerlandl?
Inferior aorta near bifurcation
73
What can falsely elevate VMA?
Coffee, tea, fruits, vanilla, iodine contrast, labetalol, alpha- and beta-blockers
74
What are ganglioneuromas?
Rare, benign, asymptomatic tumor of neural crest origin in the adrenal medulla or sympathetic chain
75
Indications for unilateral adrenalectomy?
Aldosteronoma, cortisol-secreting adenoma, unilateral pheo, virilizing or feminizing tumor, nonfunctioning tumor (>4-5cm, carcinoma, solitary unilateral mets)
76
Indications for bilateral adrenalectomy?
Bilateral pheos, Cushing's caused by bilateral nodular adrenal hyperplasia or ectopic ACTH-producing tumor unresponsive to primary therapy
77
Biochemical diagnosis of pheo?
Plastma fractionated metanephrines and/or 24h urine catecholamines and metanephrines
78
Biochemical diagnosis of aldosteronoma?
Plasma aldosterone concentration and plasma renin activity; urinary aldosterone and potassium (on high-salt diet)
79
Biochemical diagnosis of Cushing's from cortical adenoma?
24h urine free cortisol; overnight low-dose DMST; plasma ACTH
80
Biochemical diagnosis of adrenal cortical carcinoma?
24h urine cortisol, plasma DHEA level
81
Biochemical diagnosis of incidentaloma?
Low-dose DMST, plasma fractionated metanephrines/urine catecholamines; plasma aldosterone concentration and plasma renin activity if hypertensive or hypokalemic

Fiser's ABSITE Ryan SHORT (50 decks)

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