the adrenal. adrenal anatomy composed of a cortex and medulla, which have separate embryology
TRANSCRIPT
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The Adrenal
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Adrenal Anatomy Composed of a cortex and
medulla, which have separate embryology.
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Adrenal Anatomy The adrenal cortex arises fro m the
coelomic mesoderm between the fourth and sixth weeks of gestation.
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Adrenal Anatomy The adrenal medulla is derived from cells of
the neural crest that also form the sympathetic nervous system and the sympathetic ganglia.Some of these neural crest cells migrate into the adrenal cortex to form the adrenal medulla, but chromaffin tissue may also develop in extraadrenal sites.
The most common site of extraadrenal chromaffin tissue is the organ of Zuckerkandl, located adjacent to the aorta near IMA.
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Adrenal Anatomy The glands weigh about 4g each,
located in the retroperitoneum along the superior-medial aspect of the kidneys.
Yellow appearance because of their high lipid content.
3-5 cm in length, 4-6mm in thickness
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Adrenal Glands (Normal)
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Adrenal Anatomy Left>right Receive arterial blood from branches of the
inferior phrenic artery, aorta, and renal arteries.
The right adrenal vein is short and exits the gland medially to enter the vena cava. The left adrenal vein exits anteriorly and usually drains into the left renal vein. As a result, adrenal venous catheterization is accomplished more easily on the left than the right.
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Adrenal Anatomy The adrenal cortex is composed of
three zones histologically. Outer zona glomerulosa, site for
aldosterone synthesis. Central zona fasciculata and
inner zona reticularis produce both cortisol and androgens.
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Adrenal Anatomy Most of the blood supply to the
medulla comes from venous blood draining through the cortex. This provides the adrenal chromaffin cells with high concentration of the enzyme phenyethanolamine N- methyltransferase (PNMT) required for conversion of norepinephrine to epinephrine.
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The Cortex Three major hormones Cortisol Androgens Aldosterone
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The Cortex Zona glomerulosa is the
exclusive site of production of aldosterone because it lacks the enzyme 17 alpha hydroxylase necessary for production of 17 a- progesterone and 17 a-pregnalone, which are the precursors to cortisol and androgens.
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The Cortex Zona fasciculata and reticularis
function as a unit to produce cortisol, androgens, and small amounts of estrogen, but it lacks the enzymes necessary to convert 18-hydroxycorticosterone to aldosterone.
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Cortex Cholesterol is the precursor from
which all adrenal steroids are synthesized.
Conversion of cholesterol to pregnenolone is the rate limiting step in adrenal steroidogenesis and is the major site of action of ACTH.
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SteroidogenesisCholesterol
Pregnenolone
Progesterone
11-Deoxycorticosterone
Corticosterone
Aldosterone
17-Hydroxypregnenolone
17-Hydroxyprogesterone
11-Deoxycortisol
Cortisol
DHEA
Androstenedione
Testosterone
Estradiol
3β hydroxysteroid dehydrogenase
21β-hydroxylase
11β-hydroxylase
Aldosterone synthase
Cholesterol desmolase
17α-hydroxylase 17,20 lyase
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Glucocorticoids Regulated by hypothalamus and
pituitary via secretion of CRH and ACTH.
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Glucocorticoids Cortisol, like ACTH is secreted in a pulsitile
manner, and plasma levels closely parallel those of ACTH. Superimposed on this is a circadian rhythm that results in peak cortisol levels in the early morning and a nadir in the late evening.
Physical and emotional stress (trauma, surgery, and hypoglycemia) increase cortisol secretion by stimulating release of CRH and ACTH from hypothalamus and pituitary respectively.
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Glucocorticoids Normal daily production of cortisol
is 10-30mg. The liver is the main site of
metabolism. Two major metabolites are 17-hydroxycorticosteroids and 17-ketosteroids, excreted in the urine.
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Glucocorticoids Metabolic effects are stimulation of
hepatic gluconeogenesis, inhibition of protein synthesis, increased protein catabolism, and lipolysis of adipose tissue.
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Glucocorticoids The increased release of AA from muscle
protein and release of glycerol and free fatty acids from fat provide the substrate for hepatic gluconeogenesis.
Also increase glycogen synthesis, peripheral uptake of glucose is inhibited, and may cause hyperglycemia and increased insulin secretion.
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Glucocorticoids Loss of collagen, impair wound
healing by inhibition of fibroblasts. Inhibit bone formation, reduce
calcium absorption by gut (steroid induced osteoporosis).
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Glucocorticoids Numerous antiinflammatory actions,
which include inhibition of leukocyte mobilization and function, decreased migration of inflammatory cells to sites of injury, decreased production of inflammatory mediators (IL-1, leukotrienes, and bradykinins).
Also essential for cardiovascular stability, as evidenced by the collapse that occurs in patients with adrenal insufficiency.
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Androgens Dehydro-3-epiandrosterone (DHEA)
and DHEA sulfate. Minimal direct biologic activity. In periphery they undergo
conversion to androgens, testosterone, and dihydrotestosterone.
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Androgens Increased in Cushing syndrome, adrenal
carcinoma, congenital adrenal hyperplasia. In adult men accounts for only 5% of
testosterone, in prepubertal boys, however, increased production may be manifested by the early development of secondary sexual characteristics and penile enlargement.
In females, manifested by acne, hirsuitism, virilization, and amenorrhea.
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Aldosterone Maintains extracellular fluid
volume and regulation of sodium and potassium.
Renin-angiotensin system regulates it.
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Aldosterone Renin is secreted by
juxtaglomerular cells of the kidney in response to decreased pressure in the renal afferent arterioles.
Decreased sodium concentration sensed by the macula densa promote renin as well.
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Aldosterone Renin is also stimulated by
hyperkalemia, and inhibited by potassium depletion.
Angiotensin II is a potent vasoconstrictor, also stimulates zona glomerulosa to secrete aldosterone.
Aldosterone then stimulates reabsorption of sodium in exchange for potassium and hydrogen ion secretion.
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Renin-Angiotensin
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Cushings Syndrome Constellation of signs and
symptoms that result from chronic glucocorticoid excess.
Most common source is iatrogenic administration of glucocorticoids.
ACTH-secreting tumors of pituitary are the most common cause of spontaneous Cushing syndrome.
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Cushing’s Syndrome, CausesEndogenous Pituitary adenoma
(Cushing’s Disease) Ectopic ACTH
production Ectopic CRH
production Adrenal adenoma
Adrenal carcinoma Adrenal hyperplasia
Exogenous Therapeutic steroids
(pills, lotions, creams)
Major depression Alcoholism
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Cushings Syndrome Pituitary Cushing, also termed Cushing
disease, accounts for 70% of all cases of Cushing syndrome.
Ectopic ACTH secreting tumors comprise 15% of all cases and associated with small cell cancers of the lung.
Primary adrenal tumors (adenomas, carcinomas) account for 15-20% of cases.
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Cushing Syndrome These patients lose diurnal variation in
cortisol levels. Elevated levels of urinary free cortisol
present in 90% of patients. Normally only 1% of cortisol excreted in urine.
Low dose dexamethasone suppression test will suppress pituitary secretion of ACTH and adrenal production of steroids. So, if am plasma cortisol is suppressed then Cushing is ruled out.
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Cushing Syndrome Plasma ACTH levels are used to
differentiate ACTH-dependent (pituitary and ectopic ACTH secreting tumors) from adrenal causes of Cushing syndrome.
With primary adrenal tumors, ACTH should be suppressed (<5pg/ml).
With pituitary causes, it will be normal or slightly elevated (15-200pg/ml).
With ectopic ACTH secreting tumors, it will be markedly elevated.
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Cushing Syndrome High Dose Dexamethasone Test
may be used to distinguish pituitary from non-pituitary causes of ACTH-dependent Cushing syndrome.
Rationale is that the high dose will not suppress cortisol production from a primary adrenal neoplasm or ectopic ACTH secreting tumor.
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Suspect Cushing Syndrome
24 hour urine free Cortisol X 3 days 100mg/24 hr.
Cushing’s Syndrome
Low dose Dexamethasone suppression test
Equivocal (possible pseudo-Cushing’s)
No suppressionof plasma cortisol
SuppressesPlasma cortisol(<5 ng/ml)
Cushing’s Syndrome No cushing’s
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Cushing’s Syndrome
Late-afternoon/midnight measurement of plasma cortisol + ACTH
Plasma cortisol >50ng/mlPlasma ACTH <5 pg/ml
ACTH-independentCushing’s
Adrenal tumor orhyperplasia
Adrenal CT/MRI
Surgical removal
No Cushing’s
Plasma cortisol >50 ng/ml, ACTH >50 pg/ml
ACTH-dependant Cushings Syndrome
High dose dexamethasone suppression test? Metyrapone stim. Test, ?inferior petrosal
Sinus sampling
>50%reduction in cortisol
Pituitary tumor (Cushing’s disease)
Pituitary CT/MRI
Pituitary surgery
cure FailurePituitary irradiation or
Bilateral adrenalectomy
<50% reduction in cortisol
Ectopic ACTH
Thoracic/abdominal CT/MRI
Treatment of primary lesionBilateral adrenalectomyNecessary occasionally
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Adrenal Insufficiency, Addisons Weight loss, anorexia 90% Nausea, vomiting 66% Weakness, tiredness, fatigue 94% GI complaints 61% abdominal pain 28% Diarrhea 18% Muscle pain 16% Salt craving 14% Hypotension, dizziness, syncope 14% Lethargy, disorientation 12%
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Adrenal Insufficiency, Causes Autoimmune Steroid withdrawal Adrenal atrophy (lymphocytic adenitis
with fibrosis) Malignant infiltration Hemorrhage Sepsis Iatrogenic (post op) Sarcoidosis, Tuberculosis
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Adrenal Insufficiency, Diagnosis Hyponatremia Hyperkalemia Azotemia Hypercalcemia 10-30% associated with other endocrine
disorders AM cortisol level, ACTH level Rapid ACTH test
0.25 mg IV cosyntropin Measure cortisol before and 60 min after Cortisol level should be >18mcg/dl at 60 min
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Adrenal Insufficiency, Treatment Acute – stress dose
dexamethasone Chronic – hydrocortisone (200-
300mg) plus fludrocortisone (.05-1.0mg/day)
ACTH stim test to establish diagnosis
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Hyperaldosteronism Primary (suppressed renin)1. Adrenal adenoma2. Adrenal carcinoma3. Bilateral hyperplasia Secondary1. Renal artery stenosis2. Edematous states (cirrhosis, renal
failure)
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Hyperaldosteronism, Diagnosis CT scan Adrenal vein sampling Urinary 18-hydroxycortisol –
elevated in adenoma. plasma hydroxycorticosterone
(overnight recumbent)- > 100 in adenoma
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Hyperaldosteronism, Diagnosis Serum K < 3.6 mEq/L Plasma renin activity (PRA) < 1
ng/ml Plasma aldosterone >22 ng/dL Urine aldosterone > 14 mcg/24hrs Urine K > 40 mEq/24 hrs Plasma aldosterone:PRA ratio > 50:1
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Hyperaldosteronism, Treatment Bilateral hyperplasia-medical,
spironolactone, amiloride. Unilateral adenoma-
adrenalectomy.
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Adrenal Medulla L-Tyrosine converted to L-DOPA converted to Dopamine converted to L-Norepinephine converted to L-Epinephrine Degrades to VMA, metanephrine,
normetanephrine
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Catecholamines Exert their effect by interaction will
cell-specific receptors. The principle physiologic effect of
alpha receptor stimulation is vasoconstriction.
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Catecholamines Two types of Beta receptors exist. Beta-1 receptors mediate inotropic
and chronotropic stimulation of cardiac muscle, whereas Beta-2 receptors induce relaxation of smooth muscle in non-cardiac tissues, including blood vessels, the bronchi, uterus, and adipose tissue.
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Pheochromocytoma 10% extraadrenal 10% bilateral 10%familial 10%children 10% malignant 10% assoc with MEN 10% present with a stroke
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Pheochromocytoma Pounding in chest (from B-1
receptor mediated increase in CO). Headaches Hands and feet become moist,
cool, and pale (from A-receptor induced peripheral constriction).
10% present in “pheocrisis” 50% found as incidentaloma.
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Pheochromocytoma Elevated BP, fever, flushing,
sweating, anxiety, feeling of doom. Most attacks are short-lived
(15min). May be precipitated by position,
stress, physical activity….
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Pheochromocytoma, Diagnosis 24hr urinary catecholamines (NE, Epi,
Dop) and metabolites (metanephrine, normetanephrine, VMA).
Plasma catecholamine or metabolites during episode.
Elevated serum epinephrine suggests pheo in medulla or Organ of Zukerkandl
NO FNA! (can precipitate hypertensive crisis).
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Pheochromocytoma, Diagnosis Localizing studies: CT, MRI, MIBG scan
Thin cut CT detects most lesions: 97% intraabdominal.
MRI: 90% pheos bright on T2 weighted scan MIBG: used for extraadrenal, recurrent,
multifocal, malignant disease. Malignant disease
Local invasion, disease outside of adrenal/paraganglionic tissue.
No histological or clinical criteria can differentiate malignant disease.
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Pheochromocytoma, Treatment Treatment is surgery Must medically optimize prior to surgery
Treat HTN Expand intravascular volume Control cardiac arrhythmias
Phenoxybenzamine (α-adrenergic antagonist) most commonly given 1-3 wks prior to OR. Other α-adrenergic antagonists, CCB, ACEI used
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Pheochromocytoma, Treatment PO salt and fluid repletion. May need β-blocker as
antiarrhythmic. Do not start until after pt α-blocked.
Metyrosine – decreases catecholamine synthesis.
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Incidentaloma Found on work up for another cause, not
on cancer workup US 0.1% CT 0.4 to 4.4% MRI
70-94% are benign and nonfunctional >3cm more likely to be functional Up to 20% may be subclinically active Increase with age, no change in sex 5-25% will increase in size by at least 1cm
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Incidentaloma Increased risk of adrenocortical
carcinoma with increasing size<4cm 2%
4.1-6cm 6% >6cm 25%
No change with age or sex
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Incidentaloma, Workup Bioclinical examination
Dexamethasone suppression test Urinary/plasma catecholamine/metanephrines Serum potassium, plasma aldosterone
concentration-plasma renin activity ratio (if hypertensive)
Rule out other malignancy Stool for occult blood CXR Mammogram
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Incidentaloma, Who Gets Surgery? Unilateral, functioning tumors. >6cm; 4-6 cm is a grey area. Rapid growth rate. Imaging not c/w benign adenoma. No surgery if workup reveals metastasis. ?Younger patients (increased lifetime
cancer risk, longer f/u, lower incidence of adrenal masses).
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Incidentaloma, Watchful Waiting <4cm, nonfunctioning tumors. CT in 3 and 12 months. If no
increase in size, no data to support further imaging.
? Periodic hormonal testing. If a tumor will start to hyperfunction, this will most likely happen in 3-4 yrs.
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Functioning mass Nonfunctioning mass
adrenalectomy >4.5 cmAtypical CTappearance
<4.5 cmBenign
appearance
History ofExtraadrenalmalignancy
adrenalectomyRepeat CT/MRI3 and 12 months
ConsiderFNA
+ FNA - FNA
adrenalectomy observeAdapted from Camerons, Current Surgical Therapy 7th ed. Pg 635