Adrenal

Question 1

Superior adrenal

Answer 1

inferior phrenic artery

Question 2

Middle adrenal

Answer 2

aorta

Question 3

Inferior adrenal

Answer 3

renal artery

Question 4

Left adrenal vein

Answer 4

drains into left renal vein

Question 5

Right adrenal vein

Answer 5

drains into IVC

Question 6

Hypothalamus - Anterior Pituitary - Adrenal Gland

Answer 6

CRH - ACTH - Cortisol

Question 7

Asymptomatic Adrenal Mass

Answer 7

1-2% of abdominal CT scans show incidentaloma (5% mets or primary adrenal tumors); benign adenomas are common; surgery indicated if > 4cm, ominous characteristics (nonhomogenous), functioning or enlarging; need to f/u every 3months for 1 year, then yearly; dx serum K, urine metaneprhines / VMA / cathecholamines, urinary hydroxycorticosteroids, plasma renin & aldosterone levels if HTN or decrease K; CXR, stool guaiac & c-scope, mammogram; cancer history with asymptomatic adrenal mass need biopsy

Question 8

Common mets to adrenal

Answer 8

Lung cancer > breast CA > melanoma > renal CA

Question 9

Adrenal Cortex

Answer 9

from mesoderm; GFR (salt, sugar, sex steroids)
Glomerulosa: aldosterone
Fasciculata: glucocorticoids
Reticularis: androgens / estrogens
no innervation to cortex
lymphatics drain to subdiaphragmatic & renal LNs

Question 10

Cortisol

Answer 10

diurnal peak at 4-6am

Question 11

Aldosterone

Answer 11

stimulates renal Na resorption & secretion of K, hydrogen ion and ammonia; secretion is stimulated by angiotensin II & hyperkalemia and to some extent ACTH

Question 12

Excess estrogens and androgens by adrenals

Answer 12

almost always cancer

Question 13

Congenital Adrenal Hyperplasia

Answer 13

enzyme defect in cortisol synthesis

Question 14

21-hydroxlase deficiency

Answer 14

most common 90%
precocious puberty in males, virilization in females
increase 17-OH progesterone leads to increase production of testosterone
salt wasting (decrease Na, increase K); hypotension
tx: cortisol, genitoplasty

Question 15

11-hydroxylase deficiency

Answer 15

precocious puberty in males, virilization in females
increase in 11-deoxycortisone (acts as mineralocorticoid)
salt saving; causes HTN
tx: cortisol, genitoplasty

Question 16

17-hydroxylase deficiency

Answer 16

ambiguous genitalia in males at birth

salt saving

Question 17

Hyperaldosteronism (Conn’s syndrome)

Answer 17

HTN 2/2 Na retention without edema; hypokalemia; weakness, polydipsia, polyuria
Primary disease (low renin):  adenoma (85%), hyperplasia (15%), ovarian tumors (rare), cancer (rare)

Question 18

Secondary Hyperaldosteronism

Answer 18

high renin; more common than primary disease

CHF, renal artery stenosis, liver failure, pregnancy, diuretics, Bartter’s syndrome (renin-secreting tumor)

Question 19

Dx hyperaldosteronism

Answer 19

urine aldosterone after salt load (will stay high); decrease serum K, increase urine K, increase serum Na, metabolic alkalosis; plasma renin activity will be low; aldosterone:renin ratio > 20
localizing studies: MRI, NP-59 scintigraphy (shows hyperfunctioning adrenal tissue, differentiates adenoma from hyperplasia; 90% accurate), adrenal venous sampling

Question 20

Tx Hyperaldosteronism

Answer 20

adenoma - resection
hyperplasia - seldom cured, increased morbidity with b/l resection, try medical tx using spironolactone, Ca channel blockers, K
if bilateral resection is performed (usually done for refractory hypokalemia) pt will need fludrocortisone

Question 21

Hypocortisolism (adrenal insufficiency, Addison’s disease)

Answer 21

#1 cause withdrawal of exogenous steroids; #1 primary disease is autoimmune disease
also caused by pituitary disease, infection, adrenal hemorrhage, adrenal metastasis, surgical resection or injury
get decrease cortisol and aldosterone, decrease serum Na, increase serum K, ACTH stim test

Question 22

Acute adrenal insufficiency

Answer 22

hypotension, fever, lethargy, abdominal pain, decrease glucose, AMS, N/V, increase K
tx: dexamethasone, fluids

Question 23

Hypercortisolism (Cushing’s syndrome)

Answer 23

most commonly iatrogenic; 24 hour urine cortisol is most sensitive test; dexamethasone suppression test

Question 24

Dexamethasone Suppression Test

Answer 24

low dose: if low cortisol then Cushing’s disease (pituitary adenoma was suppressed); if cortisol remains high measure ACTH:
if low pt has cortisol secreting tumor (i.e., adrenal tumor or adrenal hyperplasia)
if high have either ectopic ACTH or pituitary tumor that was not suppressed, give high dose overnight dexa suppresion test:
if suppresses then pituitary origin if not ectopic origin of ACTH
in 20% still cannot tell proceed with CRH test:
pituitary adenomas will increase ACTH; ectopic producers will have no change in ACTH

Question 25

Pituitary Adenoma (Cushing's disease)

Answer 25

``` #1 noniatrogenic cause of Cushing's Syndrome 70-80%; mostly microadenomas; need petrosal sampling to figure out which side; MRI can help tx: transsphenoidal approach; unresectable or residual tumors treated with XRT ```

Question 26

Ectopic ACTH

Answer 26

``` #2 noniatrogenic cause of Cushing's syndrome; most commonly from small cell lung Cancer; CT chest / abd help to localize tx: resection of primary if possible; medical suppression or bilateral adrenalectomy for inoperable lesions ```

Question 27

Adrenal Adenoma

Answer 27

``` #3 noniatrogenic cause of Cushing's syndrome; decrease ACTH; unregulated steroid production; does not suppress tx: adrenalectomy ```

Question 28

Adrenocortical Carcinoma

Answer 28

rare cause of Cushing's syndrome; bimodal distribution (before age 5 and in 5th decade); more common in females; 50% are functioning tumors (cortisol, aldosterone, sex steroids); children display virilization 90% of time (precocious puberty in boys, virilization in females) sxs: abdominal pain, weight loss, weakness 80% have advanced disease at time of dx Tx: radical adrenalectomy; debulking can help pt and prolong survival; mitotane for residual or recurrent disease 20% 5 year survival rate

Question 29

Adrenal hyperplasia

Answer 29

macro or micro; Tx: bilateral adrenalectomy

Question 30

Medical Tx for ectopic ACTH production or adrenocortical ca with residual or mets after resection

Answer 30

ketoconazole & metyrapone (inhibit steroid formation) Aminoglutethimide (inhibits cholesterol conversion) Op-DDD aka mitotane (adrenal lytic; used for mets) give steroids post op

Question 31

Adrenal Medulla

Answer 31

from ectoderm neural crest cells; cathecholamine production: tyrosine* --> dopa --> dopamine --> NE** --> epi *tyrosine hydoxylase rate limiting step **PNMT enzyme is found only in adrenal medulla only adrenal pheochromocytomas will produce epi

Question 32

Monoamine oxidase (MAO)

Answer 32

converts NE to normetanephrine and Epi to metanephrine; VMA produced from these

Question 33

Organ of Zuckerkandl

Answer 33

chromaffin body derived from neural crest located at the bifurcation of the aorta or at the origin of IMA; can be the source of pheochromocytoma or paraganglioma

Question 34

Pheochromocytoma

Answer 34

chromaffin cells, slow growing; arise from sympathetic ganglia or ectopic neural crest cells; right > left 10% rule: malignant, bilateral, in children, familial, extra-adrenal (more likely malignant) assoc with MEN IIa/b, von Recklinghausen's disease, tuberous sclerosis, Sturge-Weber disease Sxs: HTN, headache, diaphoresis, palpitations Dx: plasma metanphrines, urine metanephrines & VMA (most sensitive); MIBG scan can help localize; CT / MRI; if unable to localize still proceed with laparotomy

Question 35

Pheo pre-op

Answer 35

volume replacement, alpha blocker first (phenoxybenzamine) then B-blocker if pt has tachycardia or arrhythmias

Question 36

Pheo surgery

Answer 36

check for other tumors at resection; ligate adrenal veins first to avoid spilling cathecholamines during tumor manipulation; debulking helps symptoms in pts with unresectable disease; metyrosine inhibits tyrosine hydroxylase causing decrease synthesis of cathecholamines; should have Nipride, neo-synephrine, and anti-arrhythmic agents ready during time of surgery

Question 37

Falsely elevated VMA

Answer 37

coffee, tea, fruits, vanilla, iodine contrast, labetalol, alpha & beta blockers

Question 38

Indications for Unilateral Adrenalectomy

Answer 38

``` aldosteronoma cortisol-secreting adenoma (Cushings) unilateral pheo virilizing or feminizing tumors nonfunctioning unilateral tumor --> size > 4cm; adrenocortical ca; solitary adrenal mets ```

Question 39

Indications for Bilateral Adrenalectomy

Answer 39

- b/l pheo - Cushing's syndrome from b/l nodular adrenal hyperplasiia or ectopic ACTH-producing tumor unresponsive to primary therapy - Cushing's disease (pituitary tumor) unsuccessfully treated by surgery or radiation