Tumors of Adrenal Glands

Question 1

What is the function of the adrenal medulla?

Answer 1

Giant presynaptic nerve ending that releases catecholamines

Question 2

How common are adrenal cancers? Prognosis?

Answer 2

rare–most are metastatic at diagnosis

Question 3

What are the ssx of hormonally active adrenal cancer?

Answer 3

Hirsutism
Acne
Hypokalemia
HTN

Question 4

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

Answer 4

Vague ssx–a abdominal pain or fullness

Question 5

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

Answer 5

True

Question 6

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

Answer 6

65% localized
44% regional
7% distant

Question 7

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

Answer 7

4 cm or larger

Question 8

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

Answer 8

Bad

Question 9

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

Answer 9

• High CT attenuation values (dense)

- irregular shape

Question 10

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

Answer 10

• Cortisol level
• Metanephrines
• Androgens/estrogens

Question 11

What are the treatments for adrenal tumors? (2)

Answer 11

Surgical is curative

Palliative is advanced ds

Question 12

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

Answer 12

less than 6 cm in size

Incisions made in the flank or midline

Question 13

What organs are commonly involved with adrenal CA?

Answer 13

• Liver
• Spleen/diaphragm
• Pancreas
• Colon

Question 14

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

Answer 14

XRT not helpful

Chemo is controversial

Question 15

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

Answer 15

Iatrogenic–exogenous corticosteroids

Question 16

What are the causes of Cushing syndrome? (3)

Answer 16

• Long term steroid use
• ACTH overproducing
• Benign adrenal lesions

Question 17

What ectopic cancers produce ACTH classically?

Answer 17

Small cell lung cancer

Question 18

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

Answer 18

Check cortisol by urine or blood

If positive, then do ACTH suppression test

Question 19

What is the purpose of doing an ACTH suppression test?

Answer 19

Localize to adrenal or ectopic/pituitary source

Question 20

What is ACTH levels with an adrenal tumor?

Answer 20

Low

Question 21

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

Answer 21

High with ectopic

Lower with pituitary

Question 22

What is the best imaging modality to diagnose adrenal tumors?

Answer 22

CT of the adrenals

If pituitary, then MRI

Question 23

What is the treatment for Cushing’s tumor?

Answer 23

If adrenal, then surgery

Question 24

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

Answer 24

Supplemental steroids

Question 25

Who is most commonly affected with pheochromocytoma?

Answer 25

20-50 y/o women

Question 26

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

Answer 26

Can potentially cause a hypertensive crisis

Question 27

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

Answer 27

Hyperthyroid

Question 28

Is the HTN caused by a pheochromocytoma paroxysmal or constant?

Answer 28

Paroxysmal

Question 29

What is the average weight of pheochromocytomas?

Answer 29

100 g

Question 30

What is the most common size of pheochromocytomas?

Answer 30

2-4 cm in size

Question 31

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

Answer 31

False

Question 32

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

Answer 32

75% sustained | 25% intermittent

Question 33

True or false: orthostatic hypotension is uncommon with pheochromocytoma

Answer 33

False

Question 34

What are the four associated conditions with pheochromocytomas?

Answer 34

- NF1 - VHL ds - Tuberous Sclerosis - MEN2A/2B

Question 35

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

Answer 35

Very sensitive, pretty specific

Question 36

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

Answer 36

Pretty sensitive, but very specific

Question 37

What is the role of CTs with pheochromocytomas?

Answer 37

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

Question 38

What is the organ of Zuckerkandl?

Answer 38

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

Question 39

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

Answer 39

Alpha blockade first w/ Phenoxybenzamine 1-2 weeks prior, then beta blockers. If give beta blockers first, then over active alpha blockers

Question 40

What is the treatment for the orthostatic hypotension with pheochromocytoma?

Answer 40

IVFs

Question 41

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

Answer 41

When there is persistent tachycardia after alpha blockade Start after phenoxybenzamine and continue until operation

Question 42

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

Answer 42

Laparoscopic

Question 43

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

Answer 43

Renal vein

Question 44

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

Answer 44

Complete other surgery fast, without manipulating the tumor

Question 45

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

Answer 45

Nitroprusside and alpha/beta blockers postoperatively

Question 46

What is primary hyperaldosteronism?

Answer 46

Failure to suppress aldosterone secretion

Question 47

What is the MOA of aldosterone?

Answer 47

Maintains intravascular volume by exchanging K for Na and H

Question 48

What is Conn's syndrome?

Answer 48

Primary aldosteronism

Question 49

What are the lab findings of Primary aldosteronism?

Answer 49

Drug resistant HTN | Hypokalemia

Question 50

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

Answer 50

True

Question 51

What are the ssx of Conn's syndrome?

Answer 51

- Muscle weakness/cramps - polydipsia - Polyuria - Nocturia - HA

Question 52

What is the three tier diagnosis strategy for primary hyperaldosteronism?

Answer 52

- Initial screening - Confirmation of dx - Determine subtype

Question 53

What is the treatment for aldosterone producing adenoma?

Answer 53

Surgery

Question 54

What is bilateral idiopathic hyperaldosteronism? Treatment?

Answer 54

- idiopathic excess production of aldosterone from both adrenal glands - Medical therapy with aldosterone antagonists

Question 55

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

Answer 55

50-75% of cases

Question 56

What percent of primary hyperaldosteronism is from IHA?

Answer 56

25-50%

Question 57

What is the relative incidence of aldosterone producing carcinomas?

Answer 57

Rare

Question 58

What is the primary screening study for primary aldosteronism?

Answer 58

Aldosterone to renin ratio (will be high)

Question 59

What is the urine aldosterone test?

Answer 59

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

Question 60

What is the saline suppression test for primary hyperaldosteronism?

Answer 60

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

Question 61

What is the role of CT with primary hyperaldosteronism?

Answer 61

Determine if unilateral or bilateral

Question 62

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

Answer 62

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

Question 63

What is the treatment for APA?

Answer 63

Laparoscopic surgery

Question 64

What is the treatment for IHA?

Answer 64

Subtotal adrenalectomy

Question 65

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

Answer 65

Spironolactone

Question 66

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

Answer 66

Serum K levels revert to normal within 24-72 hours

Question 67

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

Answer 67

over 90%

Question 68

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

Answer 68

True

Question 69

What are incidentalomas?

Answer 69

Unsuspected adrenal masses on imaging for some other reason

Question 70

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

Answer 70

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

Question 71

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

Answer 71

``` BP Weight change Cushing's ssx Virilization/feminization Occult malignancy ```

Question 72

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

Answer 72

Hypercortisolism Hyperaldosteronism Pheo

Question 73

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

Answer 73

r/o pheo

Question 74

What percent of adrenal cancers are hormonally active? Inactive?

Answer 74

60% active, 40% inactive

Question 75

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

Answer 75

Less than 4 cm is not | More than 6 is suggestive

Question 76

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

Answer 76

To determine if there's mets

Question 77

How do you follow an incidentaloma?

Answer 77

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