Adrenal Tumors Flashcards

1
Q

What are the criteria for calling an adrenal tumor an incidentaloma?

A

Over 1 cm in size

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2
Q

What type of screening should you do for solid adrenal masses?

A

You should get functional testing done

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3
Q

What characterizes a benign adrenal adenoma?

A

On CT the Hounsfield units should be under 18 or CT contrast washout at 60% at 15 minute mark.

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4
Q

What are 3 primary functional adenomas?

A

Cushing’s Syndrome, Conn syndrome, and Pheochromacytoma

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5
Q

What is unique about adrenal cortical adenomas?

A

Majority are functional

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6
Q

What is the risk of malignancy by size of adrenal incidental mass?

A

< 4 cm is 2%
4-6 cm is 6%
>6 cm is 25%

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7
Q

3 key symptoms/signs for diagnosis of Conn Syndrome

A

Refractory HTN, Muscle cramping/fatigue, and profound hypokalemia if placed on diuretic.

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8
Q

What makes a tumor benign vs malignant when it comes to aldosterone secreting adenomas.

A

Benign are under 3 cm while malignant are over 3 cm.

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9
Q

What types of labs would you expect for an aldosterone tumor?

A
Na - high 
K+ - low
H+  - low (alkalosis)
Renin - low 
Aldosterone - high
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10
Q

What is the screening regimen for Conn syndrome?

A

Serum potassium (< 3) and aldosterone:renin ratio (<20:1)

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11
Q

What’s the difference between primary and secondary hyperaldosteronism

A

Primary has low renin, secondary has high renin.

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12
Q

How do you go about confirming hyper aldosterone?

A

You get urinary aldosterone excretion +/- w/sodium loading for 3 days.

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13
Q

How do you lateralize the etiology of the tumor when not clear on imaging?:

A

Get adrenal venous sampling.

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14
Q

How would you classify the position of the adrenal glands?

A

Retroperitoneal within Gerota’s fascia

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15
Q

What are the typical size and weight of adrenal glands?

A

Weight 5-7 grams

Size 4-6cm by 2-3 cm

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16
Q

From what specific origin does the adrenal cortex arise?

A

It arises from the intermediate mesoderm of the urogenital ridge

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17
Q

When does the adrenal cortex arise?

A

It arises between 5-8 weeks

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18
Q

When it the function of the adrenal cortex?

A

Purely endocrine in function

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19
Q

What are the 3 zones within the Adrenal Cortex and what do they do ?

A

GFR
Glomerulosa - salty (aldosterone/mineral corticoids)
Fasciculata - sweet (glucocorticoids/cortisol)
Reticularis - sex (estrogens/androgens)

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20
Q

From where does the adrenal medulla hail?

A

Comes from the neural crest cells that are adjacent to the sympathetic ganglia

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21
Q

When does the adrenal medulla arise?

A

It arises in the 9th week

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22
Q

How much of the gland does the medulla account for? What cells compose it?

A

10 percent of the gland, and chromaffin cells

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23
Q

What innervation level innervates the chromaffin cells in the medulla?

A

T11-12

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24
Q

What hormones get secreted from the medulla?

A

Catecholamines like epinephrine, norepinephrine, and dopamine

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25
Q

From what amino acid are catecholamines derived from

A

Tyrosine

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26
Q

When autopsies are done, what percent of individuals are found to have incidentalomas?

A

9% in autopsy series

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27
Q

What percentage of adrenal incidentalomas are deemed to be metabolically active?

A

20% roughly

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28
Q

Patient comes in with an adrenal incidentaloma, what are your first two steps? What do you do if they are hypertensive too ?

A

First step is to do a History and Physical including BP and HR
Second step is to do a 1mg overnight dexamethasone suppression test, plasma metanephrines, or 24 hour urine catecholamine test.
If patient is hypertensive you want to do a plasma renin activity and aldosterone renin ratio test too

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29
Q

Patient with an adrenal incidentaloma is found to be non-functional, what are your next 2-3 steps?

A

If its nonfunction, assess size

  1. If over 5cm, surgical resection
  2. If under 5cm then imaging characteristics
  3. If suspicion –> FNA/Biopsy and possible resection
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30
Q

What happens if you have an adrenal incidentaloma and it’s under 5cm and looks benign on imaging?

A

Re-image at 6 months or 12 months with biochemical assessment yearly for 4 years –> if it grows over 1cm or is functional, resect.

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31
Q

Patient comes in with adrenal incidentaloma, is found to be functional, what are your next 2 steps?

A

Confirmation test followed by resection

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32
Q

When would you not get biochemical evaluation of an adrenal lesion?

A

If it’s an obvious myelolipoma

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33
Q

What makes an obvious myelolipoma on imaging?

A

Low CT attenuation of -10 to -20 Hounsfield units or presence of microscopic fat.

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34
Q

What are your 3 options for testing for cortisol hypersecretion?

A
  1. 1mg dexamethasone suppression test (OST)
  2. Late night salivary cortisol test (SCT)
  3. 24 hour urinary free cortisol evaluation
35
Q

How does salivary cortisol test compare to dexamethasone suppression test?

A

Salivary is easier for patients but suppression test is more sensitive in detecting subclinical Cushing syndrome

36
Q

How does the 1mg dexamethasone suppression test work?

A

Give 1mg at 23:00 the night prior and check morning serum cortisol levels at 08:00 - You have a putative diagnosis of Cushing Syndrome if serum cortisol levels are greater than 5 micrograms/dL

37
Q

What is the gold standard for diagnosis of Cushing syndrome?

A

24 hour urinary free cortisol is the gold standard but is more time intensive and inconvenient. Also likely higher chance of lab error if not collected properly.

38
Q

What percentage of folks will have excess aldosterone and have refractory hypertension ?

A

10% of folks

39
Q

What are the classic 3 symptoms of conn syndrome? How often do folks have all 3 ?

A

Classically hypertension, hypokalemia and alkalosis. 40% of folks will have normal potassium though.

40
Q

What’s your first test when wanting to rule out Conn syndrome?

A

You want an Aldosterone to Renin ratio in the morning.

41
Q

What is a positive aldosterone to renin ratio? What else is needed to confirm diagnosis of primary hyperaldosteronism

A

Over 20 WITH concomitant aldosterone concentration over 15 ng/mL suggest primary hyperaldosteronism

42
Q

If you want an accurate Aldosterone to Renin ratio what 2 drugs need to be stopped and how long before the test.

A

Need to stop K+ sparring diuretics and Mineralocorticoid receptor blockers 6 weeks before testing.

43
Q

Let’s say you get a positive aldosterone : renin ratio, what’s your next test?

A

Next test should be 24 hour urine study with salt loading

44
Q

What do you always need to do if you have positive aldosterone hypersecretion?

A

Test laterality by adrenal venous sampling.

45
Q

What percentage of patients with adrenal incidentalomas have pheochromocytomas?

A

4-5 percent of these patients will have PHEs

46
Q

Patient has a solid adrenal mass, what are your options for testing for PHE biochemically?

A

You can do a plasma free metanephrines and normetanephrine level and you can also do 24 hour urinary metanephrines and fractional catecholamine.

47
Q

Which is more sensitive and why? Metanephrines testing vs catecholamine?

A

Metanephrines because it’s metabolically synthesized continuously vs in pulses.

48
Q

What 5 classes of drugs need to be reviewed as these can falsely elevate catecholamine levels?

A

Levodopa, monoamine oxidase inhibitors, benzodiazepines, tetracycline and rapid withdrawal from clonidine.

49
Q

What folks need genetic testing when it comes to pheochromocytoma?

A

Those under 50, family history, extra-adrenal pheochromocytoma

50
Q

What genetic testing is warranted when you are working up PHE patients that meet criteria?

A

Look for the RET gene, VHL, or succinate dehydrogenase genes

51
Q

What percentage of folks will have adrenocortical carcinoma within the adrenal incidentaloma?

A

3 percent of folks

52
Q

Should you do testing for excess sex hormones if you find an adrenal incidentaloma?

A

No, unless you suspect adrenocortical carcinoma (62-79% have elevated sex hormones), obvious virilization, and over 5 cm in mass

53
Q

What sex hormones will you work up if they meet criteria and they have an adrenal incidentaloma ?

A

Test for DHEA, and 17-ketosteroids. If they are a woman with virilization you should get testosterone and 17beta estradiol if a man and has feminization.

54
Q

What is the goal of imaging in the workup of adrenal masses?

A

Differential between an adrenal adenoma, carcinoma, pheochromocytoma, and metastatic lesions

55
Q

What imaging can help diagnose an adrenal adenoma?

A

Lipid density on non-contrast CT
In phase and out phase on MRI
contrast washout kinetics on contrast enhanced CT

56
Q

What attenuation on non-contrast CT are considered adenomas?

A

Below 10 HU on non-contrast CT

57
Q

What does washout kinetics look like for a benign adrenal adenoma, how is this done?

A

Looks like more than 50% washout on delayed scan while not on metastatic lesions, carcinomas and PHE

You get a CT immediately after IV contrast and then again after 10-15minutes

58
Q

How do you diagnose PHE on CT

A

They will measure greater than 10 HU on unenhanced CT and will enhance greater than 100 HU on contrast studies. They will often have a well circumscribed appearance with or without a necrotic or cystic element.

59
Q

How do you diagnose PHE on MRI?

A

On MRI you get a classic light bulb signal on T2. Not as sensitive as previously thought.

60
Q

What additional testing can you do if you suspect an extra-adrenal location?

A

Meta-iodonbenzylguanidine scan

61
Q

How do you diagnose an adrenocortical carcinoma on CT? What about the HU ?

A

Usually larger than 4cm with heterogenous appearance on CT with calcifications and necrosis often present.

HU on non-contrast CT are usually greater than 25 with less than 50% washout at 10min post-contrast imaging.

62
Q

How do you tell the difference between a lipid poor adenoma and an adrenal carcinoma?

A

Lipid poor adenoma will have HU between 20-40 but washout over 50% whereas adrenal carcinomas washout is always under 50%

63
Q

Difference between an adrenal mass under 4cm to that of one over 6cm?

A

Under 4cm is only a 2% chance of being an adrenal carcinoma but over 6cm it’s over 25%.

64
Q

What should you do for all adrenal masses between 4-6cm ?

A

Remove unless clearly a myelolipoma

65
Q

What should you do about functional masses that cause Cushing’s, conn or are pheochromocytomas?

A

Adrenalectomy to avoid long term sequelae of hormonal imbalance or risk of metastasis

66
Q

What should you do if a patient has Cushing’s but is not a candidate for surgery?

A

They should get either aminoglutethimide, metyrapone or ketoconazole while watching for adrenal insufficiency

67
Q

What if a patient has subclinical Cushing syndrome, what should you do?

A

Only do surgery if worsening HTN, abnormal glucose tolerance, or osteoporosis.

68
Q

What do you do for those with primary hyperaldosteronism?

A

Adrenalectomy with adrenal venous sampling to locate the functionality of the side. Especially in case of bilateral adrenal hyperplasia.

69
Q

What should you give patients prior to an adrenalectomy?

A

Mineralcorticoid receptor antagonist with antihypertensive medication and restore potassium levels

70
Q

What can be used to predict the resolution of antihypertensive medications in patients following an adrenalectomy?

A

You can use the Aldosteronoma Resolution score

71
Q

How should you treat patients with Aldosterone secreting tumors who are not surgical candidates?

A

Spironolactone or eplerenone (latter has less side effects because it is a corticosteroid based competitive mineralocorticoid receptor antagonist with little androgen activity

72
Q

What are the hypertension presentation of PHE patients?

A

30-50 percent come with paroxysmal HTN and the rest sustained.

73
Q

Common clinical manifestation of PHE patients?

A

HTN, pallor, headache, sweating, and palpitations

74
Q

How do you know if a pheochromocytoma is malignant or not?

A

To be malignant it has to have extracapsular invasion into adjacent structures or distant metastatic disease.

75
Q

What are the 5 most common sites where you get Mets from a pheochromocytomas?

A

Bone, Lung, Liver, Regional Lymph Node, and Peritoneum.

76
Q

What is the operative mortality of pheochromocytoma resection?

A

Just under 3 percent

77
Q

What should you start before surgery for a PHE?

A

7-14 days before give alpha blocker like phenoxybenzamine, then add beta blockade with atenolol or metoprolol if they become tachycardia or develop an arrhythmia.

78
Q

What are alternatives to alpha blockade preoperatively for PHE?

A

You can try calcium channel blockade.

79
Q

What about fluids for PHE patients?

A

They need intra-vascular expansion due to the chronic vaso-constricted state.

80
Q

Intra-operatively your patient develops a hypertensive spike during a PHE resection, what are your 4 options?

A

Nitroprusside, Nicardipine, Nitroglycerine, or Phentolamine

81
Q

Patient with PHE intraoperatively develops a tachyarrhythmia what are your options?

A

Esmolol or Lidocaine

82
Q

What happens once you ligate the adrenal vein?

A

Large hypotension so you will need fluids and an alpha agonist.

83
Q

What’s the follow up following a PHE resection?

A

Lifelong due to a 16% recurrence rate. So at least annual follow up.

84
Q

Gold standard surgery for adrenal carcinoma?

A

Open adrenalectomy to allow en bloc excision