Chapter 21 - Adrenal Flashcards Preview

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Flashcards in Chapter 21 - Adrenal Deck (81)
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1
Q

Arterial supply of the adrenal?

A

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

2
Q

Venous drainage of adrenal?

A

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

3
Q

What % of CT scans show incidentaloma?

A

1-2%

4
Q

When is surgery indicated for incidentaloma?

A

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

5
Q

How often do you need to follow an incidentaloma?

A

Q3 months for 1 year, then yearly

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

7
Q

What are common mets to the adrenal?

A

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

8
Q

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

A

Biopsy

9
Q

Adrenal cortex layers and products?

A

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

10
Q

Adrenal medulla receives innervation from what?

A

Splanchnics

11
Q

What causes the release of cortisol from the adrenal cortex?

A

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

12
Q

When does cortisol peak?

A

Diurnal, 4-6am

13
Q

What are the effects of aldosterone?

A

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

14
Q

What stimulates secretion of aldosterone?

A

Angiotensin II, hyperkalemia, ACTH

15
Q

What 3 deficiencies cause congenital adrenal hyperplasia?

A

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

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

17
Q

Treatment for congenital adrenal hyperplasia?

A

Cortisol, genitoplasty

18
Q

Characteristics of 11-hydroxylase deficiency?

A

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

19
Q

Characteristics of 17-hydroxylase deficiency?

A

ambiguous genitalia in males at birth; salt saving

20
Q

Symptoms of hyperaldosteronism (Conn’s syndrome)?

A

HTN without edema, hypokalmia, weakness, polydipsia, polyuria

21
Q

Causes of primary hyperaldosteronism (low renin)?

A

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

22
Q

Causes of secondary hyperaldosteronism (high renin)?

A

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

23
Q

What is Bratter’s syndrome?

A

Renin-secreting tumor

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

25
Q

What is the NP-59 scintigraphy?

A

Shows hyper functioning adrenal tissue, differentiates adenoma from hyperplasia

26
Q

Treatment for adenoma causing hyperaldosteronism?

A

Adrenalectomy

27
Q

Treatment for hyperplasia causing hyperaldosteronism?

A

Medical tx 1st with spironalactone, ca-channel blocker, potassium

28
Q

What is the #1 cause of hypocortisolism (Addison’s disease)?

A

Withdrawal of exogenous steroids

29
Q

What is the #1 priamry disease causing hypocortisolism?

A

Autoimmune disease

30
Q

Hormone/electrolyte aberrations with hypocortisolism?

A

Low cortisol and aldosterone; low serum Na, high serum K

31
Q

Symptoms of acute adrenal insufficiency?

A

Hypotension, fever, lethargy, abdominal pian, low glucose, depressed mental status, nausea/vomiting, high K+

32
Q

Treatment of adrenal insufficiency?

A

Dexamethasone, fluids, ACTH stim test

33
Q

Most common cause of hypercortisolism (Cushing’s syndrome)?

A

Iatrogenic

34
Q

How is the diagnosis of hypercortisolism made?

A

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
Q

1 noniatrogenic cause of Cushing’s syndrome?

A

Pituitary adenoma (70-80%)

36
Q

Test findings with pituitary adenoma?

A

Cortisol should be suppressed with either low- or high-dose dexamethasone suppression test

37
Q

Treatment of pituitary adenoma?

A

Transsphenoidal resection; unresectable or residual tumors treated with XRT

38
Q

2 noniatrogenic cause of Cushing’s syndrome?

A

Ectopic ACTH - most commonly from small cell lung cancer

39
Q

Test findings with ectopic ACTH?

A

Cortisol is NOT suppressed with either low-or high-dose dexamethasone suppression test

40
Q

Treatment of ectopic ACTH secreting tumor?

A

Resection of primary if possible; medical suppression or bilateral adrenalectomy for inoperable lesions

41
Q

3 noniatrogenic cause of Cushing’s syndrome?

A

Adrenal adenoma

42
Q

Test findings with adrenal adenoma causing Cushing’s?

A

Low ACTH, unregulated steroid production; does not suppress

43
Q

Treatment for adrenal hyperplasia?

A

Bilateral adrenalectomy

44
Q

Medical therapy for ectopic ACTH production or adrenocortical cancer with residual or metastatic disease after resection?

A

Ketoconazole/metyrapone (inhibit steroid fomation), aminoglutethimide (inhibits cholesterol conversion), Op-DDD (adrenal-lytic, used for metastatic disease)

45
Q

Postoperative care of bilateral adrenalectomies includes what?

A

Steroids

46
Q

Age distribution of adrenocortical carcinoma?

A

Bimodal (before age 5, in the 5th decade)

47
Q

% of adrenocortical carcinomas that are functional?

A

50% cortisol, aldosterone, sex steroids

48
Q

Symptoms seen in children with adrenocortical carcinoma?

A

90% with virilization (precocious puberty in boys, virilization in females)

49
Q

Symptoms of adrenocortical carcinoma?

A

Abdominal pain, weight loss, weakness

50
Q

Treatment of adrenocortical carcinoma?

A

Radical adrenalectomy; mitotane for residual or recurrent disease

51
Q

Survival rate of adrenocortical carcinoma?

A

20% 5-year survival

52
Q

What is the adrenal medulla derived from?

A

Ectoderm neural crest cells

53
Q

Steps of catecholamine production?

A

Tyrosine –> dopa –> dopamine –> norepinephrine –> epinephrine

54
Q

What is the rate-limiting step in catecholamine production?

A

Tyrosine hydroxylase (tyrosine to dopa)

55
Q

What enzyme converts norepi to epi (requires methylation)?

A

PNMT, found only in the adrenal medulla

56
Q

What is the only tumor that will produce epinephrine?

A

Adrenal pheochromocytomas

57
Q

What enzyme converts noepi to normetanephrine and epi to metanephrine?

A

Monoamine oxidase (MAO)

58
Q

Where is extra0adrenal neural crest tissue found?

A

In the retroperitoneum, organ of Zuckerkandl

59
Q

What do pheochromocytomas arise from?

A

Chromaffin cells; arise from sympathetic ganglia or ectopic neural crest cells

60
Q

What is the 10% rule with pheos?

A

10% malignant, bilateral, in children, familial, extra-adrenal

61
Q

What syndromes are pheos associated with?

A

MEN IIa, MEN IIb, von Recklinghausen’s disease, tuberous sclerosis, Sturge-Weber disease

62
Q

Which side has more pheos?

A

Right side

63
Q

Are extra-adrenal tumors more likely malignant or benign?

A

Malignant

64
Q

Symptoms of pheos?

A

HTN (episodic), headache, diaphoresis, palpitations

65
Q

How is the diagnosis of pheo made?

A

Urine mtanephrines and VMA (VMA most sensitive), MIBG scan (norepi analogue) to localize, clonidine suppression test (tumor does not respond, keeps catecholamines high)

66
Q

Is venography used for pheos?

A

NO, can cause hypertensive crisis

67
Q

Preoperative treatment of pheos?

A

Volume replacement, alpha-blockers (phenoxybenzamine), then beta-blocker if pt has tachycardia or arrhythmias

68
Q

What causes hypertensive crisis with pheos?

A

Unopposed alpha stimulation; can be precipitated by beta blockers given before alpha blockers; can cause heart failure in patients with cardiomyopathy

69
Q

Treatment of pheo?

A

Resection, check for other tumors, ligate adrenal veins to avoid spilling catecholamines; debulking if unresectable; metyrosine (inhibits tyrosine hydroxylase)

70
Q

What are frequent post op conditions following pheo resection?

A

Persistent hypertension, hypotension, hypoglycemia, bronchospasm, arrhythmias, intracerebral hemorrhage, CHF, MI

71
Q

What are extra-adrenal sites of pheos?

A

Vertebral bodies, bladder, aortic bifurcation

72
Q

Where is the organ of Zuckerlandl?

A

Inferior aorta near bifurcation

73
Q

What can falsely elevate VMA?

A

Coffee, tea, fruits, vanilla, iodine contrast, labetalol, alpha- and beta-blockers

74
Q

What are ganglioneuromas?

A

Rare, benign, asymptomatic tumor of neural crest origin in the adrenal medulla or sympathetic chain

75
Q

Indications for unilateral adrenalectomy?

A

Aldosteronoma, cortisol-secreting adenoma, unilateral pheo, virilizing or feminizing tumor, nonfunctioning tumor (>4-5cm, carcinoma, solitary unilateral mets)

76
Q

Indications for bilateral adrenalectomy?

A

Bilateral pheos, Cushing’s caused by bilateral nodular adrenal hyperplasia or ectopic ACTH-producing tumor unresponsive to primary therapy

77
Q

Biochemical diagnosis of pheo?

A

Plastma fractionated metanephrines and/or 24h urine catecholamines and metanephrines

78
Q

Biochemical diagnosis of aldosteronoma?

A

Plasma aldosterone concentration and plasma renin activity; urinary aldosterone and potassium (on high-salt diet)

79
Q

Biochemical diagnosis of Cushing’s from cortical adenoma?

A

24h urine free cortisol; overnight low-dose DMST; plasma ACTH

80
Q

Biochemical diagnosis of adrenal cortical carcinoma?

A

24h urine cortisol, plasma DHEA level

81
Q

Biochemical diagnosis of incidentaloma?

A

Low-dose DMST, plasma fractionated metanephrines/urine catecholamines; plasma aldosterone concentration and plasma renin activity if hypertensive or hypokalemic