B5.034 - CBCL Prework 1 Adrenal Incidentaloma

Question 1

what are the 3 layers of the adrenal cortex

Answer 1

zoma glomerulosa zona fasciculata zona reticularis

Question 2

what is an adrenal incidentaloma

Answer 2

an adrenal mass >1cm discovered on accident

Question 3

prevalence and characteristics of adrenal incidentalomas

Answer 3

4-6% increases with age most clinically non hyper-secreting benign 5% - cortisol producing 5% - pheos 1% - aldosterone producing 4% - carcinomas 2.5% - metastatic

Question 4

primary symptoms of pheo

Answer 4

triad of headache, sweating, palpitations 1/2 have sustained HTN most of the remainder have paroxysmal spells

Question 5

what is a pheo

Answer 5

a tumor that secretes excess catecholamines

Question 6

rule of 10

Answer 6

for pheochromocytoma 10% bilateral, extraadrenal, above diaphragm, familial, malignant

Question 7

lab Dx for pheo

Answer 7

elevated plasma fractionated metanephrines elevated urinary fractionated catecholamines, metanephrines, VMA presence of adrenal mass on CT

Question 8

special techniques for dx of pheo

Answer 8

MIBG scan, pentetreotide, PET scan

Question 9

treatment of pheo

Answer 9

surgical removal with pre op alpha blockers, followed by beta blockesr volume replacement

Question 10

Answer 10

pheochromocytoma

Question 11

Answer 11

pheo

note: lack of golgi and dark rings around cell

Question 12

pathophysiology of cushings

Answer 12

excessive glucocorticoid exposure

Question 13

clinical findings of cushings

Answer 13

truncal obesity

IGT/Diabetes ~2.5%

HTN

Hyperlipidemia

Coagulopathy

Osteoporosis

depression

hypogonadism

purple abdominal striae

proximal muscle weakness/wasting

central hypothyroidism

decreased growth velocity in children

Question 14

how do you diagnose cushings

Answer 14

24 hr urinary cortisol >3x normal

Exogenous dexamethasone substitutes for endogenous cortisol in suppressing ACTH release

Late evening salivary cortisol

Question 15

describe the dexamethasone test

Answer 15

1mg of dexamethasone at 11 PM to 12 AM and measurment of serum cortisol at 8 AM, should be less than 1.8

Question 16

when do you do a pituitary MRI

Answer 16

after bichemical testing has confirmed cushings

Question 17

pituitary MRI findings

Answer 17

a discrete adenoma will be visible in 35-65% of patients

10% of the population ages 20-40 have incidental tumors of pituitary

15% of pts with ectopic ACTH have abdnormal MRI

Question 18

when do you refer a pt with cushings to surgery

Answer 18

if there is an unequivocal pituitary neoplasm on MRI >6mm in a patient without ectopic ACTH features

Question 19

what is IPSS

Answer 19

Inferior petrosal sinus sampling

Bilateral simultaneous inferior petrosal sinus and peripheral venous sampling for ACTH levels before and after CRH stimulation

Compare rations of ACTH, if ration of sinus: periphery >3 its cushings disease

This is because it shows theres a tumor on the pituitary causing this, not carcinoid in the lung or something else

Question 20

treatment of cushings

Answer 20

transphenoidal surgery to remove adenoma if pituitary

ketoconazole is an option if sugery is not

Question 21

what is ketoconazole

Answer 21

non selective inhibitor of the adrenal and gonadal steroids

drug blocks multiple enzymes in cortisol pathway, can cause headaches, sedation and nausea, hepatotoxicity

Question 22

management of cushings syndrome

Answer 22

adrenal adenoma - resecton

pituitary adenoma - transphenoidal resection

Ectopic ACTH - resection of tumor

Adrenal carcinoma - resection and chemo

Question 23

what do you do if all options for cushing synrome fail

Answer 23

bilateral adrenalectomy

Question 24

causes of aldosterone excess

Answer 24

primary aldosterone excess - high aldosterone, low renin

due to adrenocortical adenoma (conns)

bilateral idiopathic hyperaldosteronism

Question 25

describe secondary hyperaldosteronism and causes

Answer 25

high aldosterone, high renin

renovascular hypertension, diuretic use, renin secreting tumors, LVHF

Question 26

clinical features of hyperaldosteronism

Answer 26

HTN

hypokalemia, nocturia, muscle cramps, occasional severe muscle weakness, tetany, parasthesia

Question 27

who gets screened for hyperaldosteronism

Answer 27

people with HTN and hypokalemia

HTN resistant to Rx or requiring multiple meds

incidental adrenal nodule on imaging

Question 28

how do you screen for hyperaldosteronism

Answer 28

plasma aldosterone:plasma renin ration in AM

ratio >20 and plasma aldo level >15 with suppressed renin

confirm with oral salt loading or saline infusion test

or

24 hour urine for aldosterone and Na

Question 29

describe the 24hr urine test for aldosterone

Answer 29

during test urine should have a Na excretion by 200

currently cutoff for Dx is 12 mcg/day of aldosterone production

Question 30

how do you distinguish between adenoma vs bilateral adrena hyperplasia

Answer 30

adrenal imaging

if solitary unilateral >1cm in young pt under 40 do laparoscopic resection

adrenal vein sampling, if CT is normal or nodules <1cm or>40 years old, adrenal vein sampling is gold standard to determine if localized to one adrenal gland

Question 31

treatment for hyperaldosteronism

Answer 31

goal is to decrease both the blood pressure and aldosterone levels

resection will cure in 30-60% of unilateral adenomas

BP meds can work if surgery isnt an option

Question 32

what medications should be used to treat hyperaldosteronism

Answer 32

spironolactone

eplerenon

they are aldosterone receptor antagonists

Question 33

what can cause bilateral masses

Answer 33

generally not endocrine

metastatic disease

lymphoma

infection

hemorrhage

Question 34

endocrine causes of bilateral masses

Answer 34

ACTH depended cushings

bilateral pheo

bilateral primary hyperaldosteronoma