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Drafts of absite from online deck > Chapter 21: Adrenal > Flashcards

Chapter 21: Adrenal Flashcards

1
Q

vascular supply: superior adrenal

A

inferior phrenic artery

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2
Q

vascular supply: middle adrenal

A

aorta

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3
Q

vascular supply: inferior adrenal

A

renal artery

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4
Q

drainage of left adrenal vein

A

left renal vein

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5
Q

drainage of right adrenal vein

A

inferior vena cava

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6
Q

Innervation of medulla

A

sympathetic splanchnic nerve

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7
Q

innervation of adrenal cortex

A

no innervation of adrenal cortex

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8
Q

lymphatic drainage of adrenal

A

lymphatics drain to subdiaphragmatic and renal lymph nodes

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9
Q

what makes up the adrenals

A

made up of adrenal cortex and adrenal medulla

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10
Q

incidence of incidentaloma of adrenal mass on CT scan

A

1%-2% of abdominal CT scans show incidentaloma (5% are metastases)

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11
Q

two things to know about asymptomatic adrenal masses

A
  • benign adenomas are common

- adrenals are also common site for metastases

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12
Q

what to do when you see an asymptomatic adrenal mass?

A

check for functioning tumor: urine metanephrines/vma/catecholamines, urinary hydroxycorticosteroids, serum K with plasma renin and aldosterone levels
- Consider CXR, colonoscopy, mammogram to check for a primary tumor

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13
Q

when is surgery indicated for asymptomatic adrenal mass?

A

ominous characteristics (non-homogenous), is > 4-6 cm, is functioning or is enlarging

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14
Q

how do you follow an incidentaloma?

A

need repeat imaging every 3 months for 1 year, then yearly

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15
Q

approach for adrenal CA resection

A

anterior approach for adrenal CA resection

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16
Q

Common metastases to adrenal

A

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

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17
Q

Management: cancer history with asymptomatic adrenal mass

A

Need biopsy

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18
Q

what if you have isolated metastases to the adrenal gland?

A

some isolated metastases to the adrenal gland can be resected with adrenalectomy

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19
Q

layers of the adrenal cortex

A

GFR = salt, sugar, sex steroids

  • Glomerulosa: aldosterone
  • Fasciculata: glucocorticoids
  • Reticularis: androgens / estrogens
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20
Q

precursor for androgens / cortisol / aldosterone

A

cholesterol -> progesterone -> androgens/cortisol/aldosterone

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21
Q

what enzyme do all zones of the adrenal cortex contain?

A

all zones have 21- and 11-beta hydroxylase

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22
Q

Released from the hypothalamus and goes to anterior pituitary gland

A

Corticotropin-releasing hormone (CRH)

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23
Q

Released from the anterior pituitary gland and causes the release of cortisol

A

ACTH

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24
Q

Has a diurnal peak at 4-6 am

A

Cortisol

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25
Q

Inotropic, chronotropic, and increases vascular resistance; proteolysis and gluconeogenesis; decreases inflammation

A

Cortisol

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26
Q

Stimulates renal sodium resorption and secretion of potassium and hydrogen ion

A

Aldosterone

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27
Q

What stimulates aldosterone secretion?

A

Stimulates by angiotensin II and hyperkalmeia, and to some extent ACTH

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28
Q

What is significant of excess estrogens and androgens by adrenals?

A

Almost always cancer

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29
Q

Enzyme defect in cortisol synthesis

A

Congenital adrenal hyperplasia

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30
Q
  • MCC congenital adrenal hyperplasia
  • Precocious puberty in males
  • Virilization in females
  • Increased 17-OH progesterone leads to increased production of testosterone
  • Is salt wasting (decreased sodium and increased potassium) and causes hypotension
A

21-hydroxylase deficiency (90%)

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31
Q

Tx: 21-hydroxylase deficiency

A

Cortisol, genitoplasty

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32
Q

HTN secondary to sodium retention without edema; hypokalemia; also have weakness, polydipsia, and polyuira

A

Hyperaldosteronism (Conn’s syndrome)

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33
Q

1 cause of primary hyperaldosteronism

A

Adenoma (85%)

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34
Q

Causes of primary hyperaldosteronism (Conn’s syndrome)

A

Adenoma (85%), hyperplasia (15%), ovarian tumors (rare), cancer (rare)

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35
Q

Causes of secondary hyperaldosteronism (Conn’s syndrome)

A

More common than primary disease

  • CHF
  • Renal artery stenosis
  • Liver failure
  • Bartter’s syndrome (renin-secreting tumor
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36
Q

Dx for primary hyperaldosteronism

A
  • Salt load suppression test (best, urine aldosterone will stay high)
  • Aldosterone:renin ratio > 20
  • Labs: low K, high Na, high urine K, metabolic alkalosis
  • Plasma renin activity will be low
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37
Q

Localizing studies for hyperaldosteronism (Conn’s syndrome)

A

MRI, NP-59 scintigraphy (shows hyper functioning adrenal tissue; differentiates adenoma from hyperplasia; 90% accurate); adrenal venous sampling if others nondiagnostic

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38
Q

What do you need to consider for pre-op optimization in hyperaldosteronism?

A

Pre-op need control of HTN and K replacement

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39
Q

Hyperaldosteronism: adenoma tx

A

Adrenalectomy

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40
Q

Hyperaldosteronism: hyperplasia treatment

A

Seldom cured (increased morbidity with bilateral resection)

  • Try medial therapy first using spironolactone (inhibits aldosterone), calcium channel blockers, and potassium
  • if bilateral resection is performed (usually done for refractory hypokalemia), patient will need fludrocortisone postoperatively
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41
Q

Adrenal insufficiency, Addison’s disease

A

Hypocortisolism

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42
Q

1 cause hypocortisolism

A

Withdrawal of exogenous steroids

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43
Q

1 primary disease: hypocortisolism

A

Autoimmune disease

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44
Q

Causes of hypocortisolism (Addison’s disease)

A

Withdrawal of exogenous steroids (#1), pituitary disease, adrenal infection / hemorrhage / metastasis / resection

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45
Q

Causes decreased cortisol (ACTH will be high) and decreased aldosterone

A

Hypocortisolism (adrenal insufficiency, Addison’s disease)

46
Q

Dx: hypocortisolism (adrenal insufficiency, Addison’s disease)

A

Cosyntropin test (ACTH given, urine cortisol measured) - cortisol will remain low

47
Q

Hypotension, fever, lethargy, abdominal pain, nausea and vomiting, decreased glucose, increased K

A

Acute adrenal insufficiency

48
Q

Tx: acute adrenal insufficiency

A

Dexamethasone, fluids, and give cosyntropin test (dexamethasone does not interfere with test)

49
Q

Hyperpigmentation, weakness, weight loss, GI symptoms, increased K, decreased Na

A

Chronic adrenal insufficiency

50
Q

Tx: chronic adrenal insufficiency

A

Corticosteroids

51
Q
  • Hypercortisolism

- Most commonly iatrogenic

A

Cushing’s syndrome

52
Q

Most sensitive test hypercortisolism (Cushing’s syndrome)

A

24-hour urine cortisol

53
Q

1st thing to do in hypercortisolism (Cushing’s syndrome)

A

Measure 24-hour urine cortisol (most sensitive test) and ACTH

54
Q

Hypercortisolism:

  • Low ACTH
  • high Cortisol
A

Patient has a cortisol secreting lesion (eg, adrenal adenoma, adrenal hyperplasia)

55
Q

Hypercortisolism

  • High ACTH
  • High cortisol
A

Patient has a pituitary adenoma or an ectopic source of ACTH (e.g., small cell lung CA) -> move on to second part of workup (high-dose dexamethasone suppression test)

56
Q

2nd portion of work up in hypercortisolism (high ACTH)

A

If ACTH is high, give high-dose dexamethasone suppression test

  • if urine cortisol is suppressed -> pituitary adenoma
  • if urine cortisol is not suppressed -> ectopic producer of ACTH (e.g. small cell lung ca
57
Q

High dose dexamethasone suppression test:

- if urine cortisol is suppressed..

A

pituitary adenoma

58
Q

High dose dexamethasone suppression test:

- if urine cortisol is not suppressed

A

Ectopic producer of ACTH (e.g. small cell lung CA)

59
Q

Can help localize tumors and differentiate adrenal adenomas from hyperplasia

A

NP-59 scintography

60
Q

1 non-iatrogenic cause of Cushing’s syndrome

A 
Pituitary adenoma (Cushing's disease)
- 80% of cases
61
Q

How will a pituitary adenoma look on low or high dose dexamethasone suppression test?

A

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

62
Q

Pituitary adenomas: micro or maco?

A

Mostly microadenomas

63
Q

How do you figure out which side of the pituitary is affected with a pituitary adenoma resulting in hypercortisolism?

A

Need petrosal sampling to figure out which side; MRI can also help

64
Q

Tx: pituitary adenoma (Cushing’s disease)

A

Most tumors removed with transsphenoidal approach; unresectable or residual tumors treated with XRT

65
Q

2 non-iatrogenic cause of Cushing’s syndrome

A

Ectopic ACTH

66
Q

What is ectopic ACTH most commonly from?

A

Small cell lung cancer

67
Q

How will low- or high-dose dexamethasone suppression test look in ectopic ACTH production?

A

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

68
Q

How can you localize the lesion in ectopic ACTH production?

A

Chest and abdominal CT can help localize

69
Q

Tx: Ectopic ACTH

A

Resection of primary if possible; medical suppression for inoperable lesions

70
Q

3 non-iatrogenic cause of Cushing’s syndrome

A

Adrenal adenoma

71
Q

Decreased ACTH, unregulated steroid production

- Tx: adrenalectomy

A

Adrenal adenoma

72
Q

Tx: adrenal hyperplasia (macro or micro)

A

Metyrapone (blocks cortisol synthesis) and aminoglutethimide; bilateral adenalectomy if medical treatment fails

73
Q

Rare cause of Cushing’s syndrome

A

Adrenocortical carcinoma

74
Q

Consider in patients with ectopic ACTH from tumor that is unresectable (would need to be a slow growing tumor - rare) or ACTH from pituitary adenoma that cannot be found

A

Bilateral adrenalectomy

75
Q

When operating for Cushing’s syndrome what is important post op?

A

Give steroids post op when operating for Cushing’s syndrome

76
Q

1, #2, #3 causes Cushing’s disease

A
  1. Pituitary adenoma
  2. Ectopic ACTH
  3. Adrenal adenoma
77
Q

Bimodal distribution (before age 5 and in the 5th decade); more common in females

  • 50% are functioning tumors - cortisol, aldosterone, sex steroids
  • Children display virilization 90% of the time (precocious puberty in boys, virilization in females); feminization in men; masculinization in women can occur
  • Symptoms: abdominal pain, weight loss, weakness
  • 80% have advanced disease at the time of diagnosis
A

Adrenocortical carcinoma

78
Q

Tx: adrenocortical carcinoma

A

Radical adenalectomy; debulking helps symptoms, prolongs survival

79
Q

5-year survival rate adrenocortical carcinoma

A

20%

80
Q

From ectoderm neural crest cells

A

Adrenal medulla

81
Q

Pathway of catecholamine production

A

Tyrosine -> dopa -> dopamine -> norepinephrine -> epinephrine

82
Q

Rate limiting step (tyrosine to dopa)

A

Tyrosine hydroxylase

83
Q

Enzyme that is an exclusive producer of epinephrine

- Only found in the adrenal medulla

A

PNMT (phenylethanolamine N-methyltransferase)

84
Q

What does PNMT (phenylethanolamine N-methyltransferase)

A

Enzyme converts norepinephrine -> epinephrine

- Exclusively found in adrenal medulla, exclusive producer of epinephrine

85
Q

What do adrenal pheochromocytoma produce?

A

Only adrenal pheochromocytomas will produce epinephrine

86
Q

Breaks down catecholamines; converts norepinpehrine to normetanephrine, epinephrine to metanephrine

A

MAO (monoamine oxidase)

87
Q

How is VMA (vanillylmandelic acid) produced?

A

Breakdown product of catecholamines (normetanephrine and metanephrine)

88
Q

Where can you find extra-adrenal rests of neural crest tissue?

A

Usually in the retroperitoneum, most notably in the organ of Zuckerkandl at the aortic bifurcation

89
Q

What is the origin of pheochromocytoma?

A

Chromaffin cells of the adrenal medulla

90
Q
  • Rare; usually slow growing; arise form sympathetic ganglia or ectopic neural crest cells
  • Symptoms: HTN (frequently episodic), headache, diaphoresis, palpitations
A

Pheochromocytoma

91
Q

What can pheochromocytoma be associated with?

A

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

92
Q

Laterality of pheochromocytoma

A

Right sided predominance

93
Q

10% rule of pheochromocytoma

A

Malignant, bilateral, in children, familial, extra-adrenal

94
Q

What is significant of extra-adrenal tumors in pheochromocytoma?

A

Extra-adrenal tumors are more likely malignant

95
Q

Dx: pheochromocytoma

A

Urine metanephrines and VMA

  • VMA most sensitize test for diagnosis
  • MIBG scan (norepinephrine analogue) - can help identify location if having trouble finding tumor with CT scan / MRI
  • Clonidine suppression test: tumor does not respond, keeps cathetcholamines increased
  • No venography -> can cause hypertensive crisis
96
Q

Most sensitize diagnostic test in pheochromocytoma

A

VMA

97
Q

What is a MIBG scan?

A

MIBG (norepinephrine analogue) -> can help identify location if having trouble finding tumor with CT scan / MRI

98
Q

How is the clonidine suppression test useful in pheochromocytoma?

A

Tumor does not respond, keeps catecholamines elevated

99
Q

Why no venography in pheochromocytoma?

A

Can cause hypertensive crisis

100
Q

Preoperative management of pheochromocytoma

A

Volume replacement and alpha-blocker first (phenoxybenzamine -> avoids hypertensive crisis); then B-blocker if patient has tachycardia or arrhythmias)

101
Q

Why do you ALWAYS give alpha blocker before beta blocker in pheochromocytoma?

A

Can precipitate hypertensive crisis (unopposed alpha stimulation, can lead to stroke) and heart failure

102
Q

Tx: pheochromocytoma

A

Adrenalectomy -> ligated adrenal veins first to avoid spilling catecholamines during tumor manipulation

103
Q

How do you avoid spilling catecholamines during tumor manipulation in pheochromocytoma?

A

Ligate adrenal veins

104
Q

Pheo: helps symptoms in patients with unresectable disease

A

Debulking

105
Q

Inhibits tyrosine hydroxylase causing decreased synthesis of catecholamiens.

A

Metyrosine

106
Q

What meds should you have ready during the time of surgery of pheochromocytoma?

A

Nipride, Neo-Synephrine, and anti arrhythmic agents (eg, amiodarone) ready during the time of surgery

107
Q

Post op conditions to look for s/p adrenalectomy for pheochromocytoma

A

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

108
Q

Other sites of pheochromocytomas

A

Vertebral bodies, opposite adrenal gland, bladder, aortic bifurcation

109
Q

Most common site of extra medullary tissue in pheochromocytoma

A

Organ of Zuckerkandl (inferior aorta near bifurcation)

110
Q

What can cause falsely elevated VMA?

A

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

111
Q

Responsible for medullary CA of thyroid and extra-adrenal pheochromocytoma

A

Extra-medullary tissue

112
Q

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

A

Ganglioneuroma

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