Fiser.21.Adrenal Flashcards
1
Q
What is the vascular supply to the superior adrenal gland?
A
inferior phrenic artery
2
Q
What is the vascular supply to the middle adrenal gland?
A
aorta
3
Q
What is the vascular supply to the inferior adrenal gland?
A
renal artery
4
Q
where does the left adrenal vein drain to?
A
left renal vein
5
Q
where does the right adrenal vein drain to?
A
IVC
6
Q
what are the two parts of the adrenal gland?
A
adrenal cortex and the adrenal medulla
7
Q
what is the innervation of the adrenal cortex?
A
no innervation to the cortex
8
Q
what is the innervation of the adrenal medulla?
A
innervated by the sympathetic splanchnic nerves
9
Q
where do the lymphatics from the adrenals drain to?
A
subdiaphragmatic and renal LNs
10
Q
describe the anatomy surrounding the anterior approach for left adrenalectomy
A
note the position of the phrenic vein in relationship to the left adrenal vein and tumor
11
Q
describe the anatomy surrounding the anterior approach for right adrenalectomy
A
.
12
Q
what is the HPA axis for cortisol?
A
CRH released by hypothalamus –> ACTH by apit –> cortisol by adrenal gland –> negative feedback
13
Q
what percent of CT scans show an incidental adrenal mass?
A
1-2%
14
Q
what percentage of adrenal incidentalomas are mets?
A
5%
15
Q
what is the MCC of asymptomatic adrenal masses?
A
benign adenomas
16
Q
what is the workup to r/o a functional adrenal mass?
A
urine metanephrines / VMA / catecholamines; urinary hydroxycorticosteroids, serum K with plasma renin and aldosterone levels
17
Q
what is the workup to find a primary tumor if adrenal mets are suspected?
A
CXR, colonoscopy, and mammogram
18
Q
what are four indications for adrenal mass resection?
A
ominous characteristics (non-homogenous); >4-6cm; functioning; or enlarging
19
Q
how frequently do you need to image an incidentaloma to follow it as an outpatient?
A
repeat imaging every 3 months x 1 year; then yearly thereafter
20
Q
what is the MCC of cancer mets to the adrenal? (top 4)
A
1 = lung CA, then breast CA, melanoma, renal CA
21
Q
what is the workup for an asymptomatic adrenal mass in a patient with cancer history?
A
needs biopsy
22
Q
how do you treat isolated mets to the adrenal gland?
A
adrenalectomy
23
Q
what is the embryologic origin of the adrenal cortex?
A
mesoderm
24
Q
what are the three layers of the adrenal cortex and what do they produce?
A
GFR = salt, sugar, sex; glomerulosa = aldosterone; fasciculata = glucocorticoids; reticularis = androgens / estrogens
25
what is the progression from cholesterol to the end products of the adrenal gland?
cholesterol --\> progesterone --\> androgens / cortisol / aldosterone
26
what two enzymes are present in all three zones of the adrenal cortex?
21-beta-hydroxylase and 11-beta-hydroxylase
27
where is corticotropin releasing hormone released from?
hypothalamus
28
after CRH is released by the hypothalamus, where does it go?
anterior pituitary gland
29
what hormone is released by the anterior pituitary gland to act on the adrenal cortex and what does it do?
anterior pituitary gland releases ACTH, which stimulates the adrenal cortex to release cortisol
30
when does cortisol have its diurnal peak?
at 4-6am
31
what are the 3 cardiovascular effects of cortisol?
inotropic, chronotropic, increases vascular resistance
32
what are the two metabolic effects of cortisol?
proteolysis and gluconeogenesis
33
what are the inflammatory effects of cortisol?
decreases inflammation
34
what are the effects of aldosterone in the kidney?
stimulates renal sodium resorption and secretion of potassium and hydrogen ion
35
name three stimuli for aldosterone secretion
angiotensin II, hyperkalemia, ACTH
36
what should you suspect with excess estrogen and androgen secretion by adrenals?
almost always cancer
37
what is the underlying pathophysiology of congenital adrenal hyperplasia?
enzyme defect in cortisol synthesis
38
what is the MC subtype of congenital adrenal hyperplasia and how often is it seen?
21-hydroxylase deficiency is the most common and seen in 90% of cases of CAH
39
what are the sx a/w 21-hydroxylase deficiency
precocious puberty in males and virilization in females
40
what is the underlying pathophysiology / chemical reaction causing sx with 21-hydroxylase deficiency
increased 17-OH-progesterone leads to increased production of testosterone
41
is 21-hydroxylase deficiency salt wasting or salt saving?
salt wasting causing reduced sodium, increased potasssium, and hypotension
42
how do you treat 21-hydroxylase deficiency?
cortisol and genitoplasty
43
what are the sx a/w 11-hydroxylase deficiency?
precocious puberty in males or virilization in females
44
what chemical builds up in 11-hydroxylase deficiency?
increased 11 deoxycortisone
45
is 11-hydroxylase deficiency salt saving or salt wasting?
salt saving because deoxycortisone acts as a mineralocorticoid and causes hypertension
46
how do you treat 11-hydroxylase deficiency?
cortisol, genitoplasty
47
what hormone is built up in Conn's syndrome?
aldosterone, (Conn's syndrome = hyperaldosteronism)
48
what are the sx a/w Conn's syndrome?
HTN 2/2 sodium retention without edema, hypokalemia, weakness, polydypsia, polyuria
49
what is the renin level associated with primary hyperaldosteronism?
renin level is low
50
what are the causes (and frequencies) of primary hyperaldosteronism?
adenoma (85%); adrenal hyperplasia (15%), ovarian tumors (rare); cancer (rare)
51
what is the renin level associated with secondary hyperaldosteronism?
renin level is high
52
which is more common: primary or secondary hyperaldosteronism?
secondary is more common
53
what are the 5 causes of secondary hyperaldosteronism?
renal artery stenosis, CHF, liver dz, diuretics, Bartter's syndrome (renin-secreting tumor)
54
how do you diagnose primary hyperaldosteronism?
salt load suppression test --\> best test, aldosterone will stay high; aldosterone:renin ratio \> 20; plasma renin activity will be low
55
what are the BMP, UA, and ABG findings a/w primary hyperaldosteronism?
low serum potassium, high serum sodium, high urine K, metabolic alkalosis
56
why would you use NP-59 scintigraphy to localize source of primary hyperaldosteronism?
shows hyperfunctioning adrenal tissue, differentiates adenoma from hyperplasia, 90% accurate
57
what two other localizing studies are available for primary hyperaldosternism besides the NP-59 scintigraphy?
MRI or adrenal vein sampling
58
what two preoperative issues need to be controlled in primary hyperaldosteronism?
control HTN, K replacement
59
how do you treat an adenoma causing primary hyperaldosteronism?
adrenalectomy
60
how do you treat primary hyperaldosteronism 2/2 adrenal hyperplasia
seldom cured, medical therapy with spironolactone (inhibits aldosterone), CCBs, and potassium
61
what are the adverse effects a/w bilateral resection with primary hyperaldosteronism 2/2 adrenal hyperplasia
increased morbidity with bilateral adrenalectomy
62
why would bilateral adrenalectomy be indicated with primary hyperaldosteronism 2/2 adrenal hyperplasia?
refractory hypokalemia
63
what medication will patients need postop s/p bilateral adrenalectomy for treatment of primary hyperaldosteronism 2/2 adrenal hyperplasia?
fludrocortisone
64
What is the abnormality a/w Addison's disease?
hypocortisolism / adrenal insufficiency
65
what is the MCC of Addison's disease?
withdrawal of exogenous steroids
66
what is the MCC of primary Addison's disease as well as five other causes?
#1 cause = autoimmune disease; other causes include pituitary disease, adrenal infection, adrenal hemorrhage, adrenal mets, adrenal resection
67
what are the effects of Addison's disease on cortisol, ACTH, and aldosterone levels?
low cortisol, high ACTH, low aldosterone
68
how do you diagnose Addison's disease?
cosyntropin test: adminsiter ACTH, measure urine cortisol. Urine cortisol will remain low with hypocortisolism
69
name five symptoms and two BMP findings a/w acute adrenal insufficiency?
hypotension; fever; lethargy; N/V; hypoglycemia; hyperkalemia
70
how do you treat acute adrenal insuficiency?
dexamethasone, fluids, and administer cosyntropin test
71
does dexamethasone interfere with the cosyntropin test?
nope
72
name four sx and 2 BMP findings a/w chronic adrenal insufficiency
hyperpigmentation, weakness, weight loss, GI symptoms, hyperkalemia, hyponatremia
73
how do you treat chronic adrenal insufficiency?
corticosteroids
74
what is the hormonal disturbance seen with Cushing's syndrome?
hypercortisolism
75
what is the MCC of Cushing's syndrome?
most commonly iatrogenic
76
what is the first diagnostic test you perform when evaluating for Cushing's syndrome?
24 hour urine cortisol and ACTH
77
How do you interpret a patient being evaluated for Cushing's syndrome with 24 hour urine with low ACTH and elevated cortisol?
patient has a cortisol-secreting lesion (adrenal adenoma or adrenal hyperplasia)
78
How do you interpret a patient being evaluated for Cushing's syndrome with 24 hour urine with elevated ACTH and elevated cortisol? What is the next step in dx?
pt has a pituitary adenoma or an ectopic source of ACTH. Next step is to perform a high-dose dexamethasone suppression test.
79
what cancer can be an ectopic source of ACTH?
small cell lung CA
80
How do you interpret a patient with Cushing's syndrome with suppressed urine cortisol after the high-dose dexamethasone suppression test?
pituitary adenoma
81
How do you interpret a patient with Cushing's syndrome with elevated/unchanged urine cortisol after the high-dose dexamethasone suppression test?
ectopic producer of ACTH
82
why is NP-59 scintigraphy useful in Cushing's syndrome?
can localize tumors and differentiate adrenal adenomas from hyperplasia
83
What is Cushing's disease?
pituitary adenoma
84
what is the #1 cause of non-iatrogenic Cushing's disease and with what frequency?
80% of non-iatrogenic cases of Cushing's syndrome 2/2 Cushing's disease (pituitary adenoma)
85
what are the findings in Cushing's disease with low- and high-dose dexamethasone suppression test?
cortisol should be suppressed with either low- or high-dose dexamethasone suppression test
86
is Cushing's disease usually 2/2 macroadenomas or microadenomas?
microadenomas
87
what are two diagnostic maneuvers to figure out the laterality of Cushing's disease?
petrosal venous sampling or MRI
88
how do you treat Cushing's disease?
most tumors removed with transsphenoidal approach; unresectable or residual tumor tx with XRT
89
what is the #2 non-iatrogenic cause of Cushing's disease?
ectopic ACTH
90
what is the MCC of ectopic ACTH?
small cell lung CA
91
what are the findings with the low and high-dose dexamethasone suppression test with ectopic ACTH?
cortisol is not suppressed with either low or high-dose dexamethasone suppression test
92
what imaging can be used to help localize source of ectopic ACTH?
CT chest/abdomen
93
what is the treatment for ectopic ACTH?
resection of primary tumor if possible; medical suppression for inoperable lesions
94
what is the #3 cause of non-iatrogenic Cushing's syndrome?
adrenal adenoma
95
what are the ACTH findings with adrenal adenoma?
low ACTH with elevated cortisol, its unregulated steroid production
96
what is the treatment for adrenal adenoma?
adrenalectomy
97
what are the first and second-line treatments for adrenal hyperplasia?
metyrapone (blocks cortisol synthesis) and aminoglutethimide (inhibits steroid production); bilateral adrenalectomy if medical tx fails
98
what are two indications for bilateral adrenalectomy in the setting of Cushing's syndrome?
1) patients with ectopic ACTH from a tumor that is unresectable (would need to be a slow growing tumor-this is rare); or 2) ACTH from pituitary adenoma that cannot be found
99
what medication do you need to provide after bilateral adrenalectomy for Cushing's syndrome
steroids postop
100
what age groups and sex are most likely to present with adrenocortical carcinoma?
bimodal distribution (before age 5 and in the 5th decade); more common in females
101
what symptoms are seen in children with adrenocortical carcinoma?
90% show virilization - precocious puberty in boys, virilization in girls; abdominal pain; weight loss; weakness
102
what symptoms are seen in adults with adrenocortical carcinoma?
rare cause of Cushing's syndrome; feminization in men, masculinization in women; abdominal pain; weight loss; weakness
103
what percentage of patients with adrenocortical carcinoma have advanced disease at the time of diagnosis?
80%
104
what is the treatment for adrenocortical carcinoma?
radical adrenalectomy
105
what is the role of debulking in treatment of adrenocortical carcinoma?
debulking helps symptoms and prolongs survival
106
what is the role of mitotane in the treatment of adrenocortical carcinoma?
mitotane is an adrenal-lytic for residual, recurrent, or metastatic disease
107
what is the 5-year survival rate for adrenocortical carcinoma?
20%
108
what is the embryological origin of the adrenal medulla?
from ectoderm neural crest cells
109
what are the five steps of catecholamine production starting with tyrosine
tyrosine --\> dopa --\> dopamine --\> norepinephrine --\> epinephrine
110
what is the enzyme that catalyzes the rate limiting step in catecholamine production?
tyrosinehydroxylase catalyzes tyrosine --\> dopa
111
what is the enzyme that converts norepinephrine to epinephrine and where is it located?
PNMT (phenylethanolamine N-methyltransferase) located only in the adrenal medulla (exclusive producers of epinephrine)
112
what is the only tumor that will produce epinephrine?
adrenal pheochromocytoma
113
what is the function of monamine oxidase? (3)
breaks down catecholamines; converts norepinephrine to normetanephrine; epinephrine to metanephrine
114
what is the byproduct of the 3 reactions catalyzed by monoamine oxidase?
vanillylmandelic acid (VMA)
115
where are there extra-adrenal rests of neural crest tissue?
usually in the retroperitoneum, most notably in the organ of Zuckerkandl at the aortic bifurcation
116
what is the cell type that makes up pheochromocytomas?
chromaffin cells, arise from sympathetic ganglia or ectopic neural crest cells
117
how rare and fast-growing are pheochromocytomas?
rare and slow-growing
118
what is the 10% rule of pheochromocytomas (5)
10% malignant; 10% bilateral; 10% in children; 10% familial; 10% extra-adrenal
119
name 5 disease syndromes a/w pheochromocytomas
MEN-IIa; MEN-IIb; von Recklinghausen's disease; tuberous sclerosis; Sturge-Weber disease
120
what side do pheos tend to present on?
right-sided predominance
121
are extra-adrenal tumors more likely to be benign or malignant
more likely to be malignant
122
what are the S/Sx a/w pheochromocytoma
episodic HTN; headache; diaphoresis; palpitations
123
how do you dx pheochromocytoma?
urine metanephrines & VMA (VMA most sensitive); clonidine suppression test: tumor does not respond and keeps catecholamines elevated
124
how do you image pheochromocytoma that is not seen on CT/MRI?
MIBG scan (norepinephrine analogue) can help identify location if having trouble finding tumor with CT/MRI
125
why should you avoid venography in pheochromocytoma?
can cause hypertensive crisis
126
what three meds need to be given preoperatively in patients with pheochromocytoma and in what order?
1) volume replacement; 2) alpha blocker first (phenoxybenzamine) to avoid hypertensive crisis; then 3) beta blocker if patient has tachycardia or arrhythmias
127
why does the order of supplying antihypertensives to a patient with pheochromocytoma matter?
need to give alpha blocker before beta blocker because unopposed alpha stimulation can lead to stroke, hypertensive crisis, and heart failure
128
how do you treat pheochromocytoma and what do you need to do before manipulating the tumor?
treat with adrenalectomy, ligate adrenal veins first to avoid spilling catecholamines
129
what is the role of debulking in treating patients with pheochromocytoma?
debulking helps symptoms in patients with unresectabe disease
130
what is the role of metyrosine in treating pheochromocytoma?
inhibits tyrosine hydroxylase causing reduced synthesis of catecholamines
131
what three meds need to be ready at the time of surgery for pheochromocytoma?
sodium nitroprusside (Nipride); phenlyephrine (Neosynephrine); and antiarrhythmic agents (ex: amiodarone)
132
what are 9 postop complications after adrenalectomy for pheochromocytoma?
persistent HTN, hypotension, hypoglycemia, bronchospasm, arrhythmias, intracerebral hemorrhage, CHF, MI
133
name other sites of pheochromocytomas (4)
vertebral bodies, opposite adrenal gland, bladder, aortic bifurcation
134
what is the most common site of extramedullary tissue with pheochromocytomas?
organ of Zuckerkandl (inferior aorta near bifurcation)
135
name 8 causes of falsely elevated VMA
coffee, tea, fruits, vanilla, iodine contrast, labetalol, alpha blockers, beta blockers
136
name two cancers caused by extra-medullary tissue
medullary cancer of the thyroid and extra-adrenal pheochromocytoma
137
what is a ganglioneuroma (cell of origin) and where is it located?
rare, benign, asymptomatic neural crest tumor in the adrenal medulla or sympathetic chain
138
how do you treat ganglioneuroma
resection
