Tumors of Adrenal Glands Flashcards

1
Q

What is the function of the adrenal medulla?

A

Giant presynaptic nerve ending that releases catecholamines

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2
Q

How common are adrenal cancers? Prognosis?

A

rare–most are metastatic at diagnosis

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3
Q

What are the ssx of hormonally active adrenal cancer?

A

Hirsutism
Acne
Hypokalemia
HTN

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4
Q

What are the ssx of NON-hormonally active adrenal cancer?

A

Vague ssx–a abdominal pain or fullness

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5
Q

True or false: most of the adrenal cancers are metastatic at diagnosis

A

True

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6
Q

What is the survival rate of localized, regional, and distant adrenal tumors?

A

65% localized
44% regional
7% distant

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7
Q

What size of an adrenal mass on CT/MRI is concerning for adrenal CA?

A

4 cm or larger

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8
Q

Are intralesional calcifications of adrenal tumors a good or bad prognostic marker?

A

Bad

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9
Q

What CT findings are particularly concerning of adrenal tumors? (2)

A
  • High CT attenuation values (dense)

- irregular shape

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10
Q

What are the lab studies that should be obtained for adrenal cancers? (3)

A
  • Cortisol level
  • Metanephrines
  • Androgens/estrogens
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11
Q

What are the treatments for adrenal tumors? (2)

A

Surgical is curative

Palliative is advanced ds

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12
Q

What size of adrenal tumors may be taken out laparoscopically? Where on the body are these incisions made?

A

less than 6 cm in size

Incisions made in the flank or midline

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13
Q

What organs are commonly involved with adrenal CA?

A
  • Liver
  • Spleen/diaphragm
  • Pancreas
  • Colon
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14
Q

What is the role of chemo/radiation with adrenal cancer?

A

XRT not helpful

Chemo is controversial

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15
Q

What is the most common cause of Cushing’s disease?

A

Iatrogenic–exogenous corticosteroids

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16
Q

What are the causes of Cushing syndrome? (3)

A
  • Long term steroid use
  • ACTH overproducing
  • Benign adrenal lesions
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17
Q

What ectopic cancers produce ACTH classically?

A

Small cell lung cancer

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18
Q

How do you diagnose Cushing’s? (2 steps, in sequence)

A

Check cortisol by urine or blood

If positive, then do ACTH suppression test

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19
Q

What is the purpose of doing an ACTH suppression test?

A

Localize to adrenal or ectopic/pituitary source

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20
Q

What is ACTH levels with an adrenal tumor?

A

Low

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21
Q

What is the result of an ACTH suppression test with a pituitary or ectopic tumor?

A

High with ectopic

Lower with pituitary

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22
Q

What is the best imaging modality to diagnose adrenal tumors?

A

CT of the adrenals

If pituitary, then MRI

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23
Q

What is the treatment for Cushing’s tumor?

A

If adrenal, then surgery

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24
Q

What should be given to pts in the perioperative period for a cortisol producing adrenal tumor?

A

Supplemental steroids

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25
Q

Who is most commonly affected with pheochromocytoma?

A

20-50 y/o women

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26
Q

Why do you want to diagnose and treat a pheochromocytoma prior to performing any surgery?

A

Can potentially cause a hypertensive crisis

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27
Q

Who usually is affected with pheochromocytoma: hyper or hypothyroid patients?

A

Hyperthyroid

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28
Q

Is the HTN caused by a pheochromocytoma paroxysmal or constant?

A

Paroxysmal

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29
Q

What is the average weight of pheochromocytomas?

A

100 g

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30
Q

What is the most common size of pheochromocytomas?

A

2-4 cm in size

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31
Q

True or false: there is a strong correlation between pheochromocytoma size and clinical ssx

A

False

32
Q

What percent of pts with pheochromocytomas have sustained vs intermittent HTN?

A

75% sustained

25% intermittent

33
Q

True or false: orthostatic hypotension is uncommon with pheochromocytoma

A

False

34
Q

What are the four associated conditions with pheochromocytomas?

A
  • NF1
  • VHL ds
  • Tuberous Sclerosis
  • MEN2A/2B
35
Q

What is the relative sensitivity/specificity of plasma metanephrines with a pheochromocytoma?

A

Very sensitive, pretty specific

36
Q

What is the relative sensitivity/specificity of 24 hr urine catecholamines and metanephrines with a pheochromocytoma?

A

Pretty sensitive, but very specific

37
Q

What is the role of CTs with pheochromocytomas?

A

Very accurate diagnosis of ds, as well as hepatic mets and extra adrenal sites

38
Q

What is the organ of Zuckerkandl?

A

Neuroendocrine cells that form a pheochromocytoma located at the bifurcation of the aorta

39
Q

What are the 2 drugs that should be administered prior to surgery for a pheochromocytoma? Why is the order specific for this?

A

Alpha blockade first w/ Phenoxybenzamine 1-2 weeks prior, then beta blockers.

If give beta blockers first, then over active alpha blockers

40
Q

What is the treatment for the orthostatic hypotension with pheochromocytoma?

A

IVFs

41
Q

When are beta blockers needed prior to surgery for a pheochromocytoma?

A

When there is persistent tachycardia after alpha blockade

Start after phenoxybenzamine and continue until operation

42
Q

What is the most common surgery modality utilized for a pheochromocytoma?

A

Laparoscopic

43
Q

What is the key structure that, when ligated, “deactivates” a pheochromocytoma, assuming it is on the adrenal gland

A

Renal vein

44
Q

What should you do if you discover a pheochromocytoma while performing a surgery?

A

Complete other surgery fast, without manipulating the tumor

45
Q

How do you control the HTN that can occur with a pheochromocytoma if you find one incidentally in surgery?

A

Nitroprusside and alpha/beta blockers postoperatively

46
Q

What is primary hyperaldosteronism?

A

Failure to suppress aldosterone secretion

47
Q

What is the MOA of aldosterone?

A

Maintains intravascular volume by exchanging K for Na and H

48
Q

What is Conn’s syndrome?

A

Primary aldosteronism

49
Q

What are the lab findings of Primary aldosteronism?

A

Drug resistant HTN

Hypokalemia

50
Q

True or false: the majority of patients with primary hyperaldosteronism are normokalemic

A

True

51
Q

What are the ssx of Conn’s syndrome?

A
  • Muscle weakness/cramps
  • polydipsia
  • Polyuria
  • Nocturia
  • HA
52
Q

What is the three tier diagnosis strategy for primary hyperaldosteronism?

A
  • Initial screening
  • Confirmation of dx
  • Determine subtype
53
Q

What is the treatment for aldosterone producing adenoma?

A

Surgery

54
Q

What is bilateral idiopathic hyperaldosteronism? Treatment?

A
  • idiopathic excess production of aldosterone from both adrenal glands
  • Medical therapy with aldosterone antagonists
55
Q

What percent of primary hyperaldosteronism is from an aldosterone producing adenoma?

A

50-75% of cases

56
Q

What percent of primary hyperaldosteronism is from IHA?

A

25-50%

57
Q

What is the relative incidence of aldosterone producing carcinomas?

A

Rare

58
Q

What is the primary screening study for primary aldosteronism?

A

Aldosterone to renin ratio (will be high)

59
Q

What is the urine aldosterone test?

A

-24 hr urine aldosterone w/ high salt diet for 5 days

60
Q

What is the saline suppression test for primary hyperaldosteronism?

A

Saline given should suppress aldosterone, but if still high, then suspicious for hyperaldosteronism

61
Q

What is the role of CT with primary hyperaldosteronism?

A

Determine if unilateral or bilateral

62
Q

What is the role of renal vein sampling with primary hyperaldosteronism?

A

Renal sampling compares the aldosterone levels to determine the laterality of adrenal lesions
-used for when CT scans are equivocal

63
Q

What is the treatment for APA?

A

Laparoscopic surgery

64
Q

What is the treatment for IHA?

A

Subtotal adrenalectomy

65
Q

What is the drug that is used to treat primary hyperaldosteronism medically?

A

Spironolactone

66
Q

What are the post-op results with primary hyperaldosteronism treatment?

A

Serum K levels revert to normal within 24-72 hours

67
Q

What percent of HTN is cured with surgery for primary hyperaldosteronism?

A

over 90%

68
Q

True or false: malignancy is extremely rare with primary hyperaldosteronism?

A

True

69
Q

What are incidentalomas?

A

Unsuspected adrenal masses on imaging for some other reason

70
Q

What are the three questions that should always be asked with incidentalomas?

A
  • Is it functional
  • What is the malignant potential
  • Does the pt have a primary CA, and could this be a met
71
Q

What are the pertinent history bits to obtain with adrenal incidentalomas?

A
BP
Weight change
Cushing's ssx
Virilization/feminization
Occult malignancy
72
Q

What are the lab tests that should be ordered with an incidentaloma?

A

Hypercortisolism
Hyperaldosteronism
Pheo

73
Q

What must always be checked prior to performing an FNA on an adrenal mass?

A

r/o pheo

74
Q

What percent of adrenal cancers are hormonally active? Inactive?

A

60% active, 40% inactive

75
Q

What are the sizes of incidentalomas that suggest CA, vs not?

A

Less than 4 cm is not

More than 6 is suggestive

76
Q

When is an FNA helpful in the diagnosis of an adrenal mass?

A

To determine if there’s mets

77
Q

How do you follow an incidentaloma?

A

Serial CT every 3-6 months. No need for additional functional testing iff there are no ssx