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ENRP Exam 2 > Pathology of the Adrenal Glands- Hillard > Flashcards

Pathology of the Adrenal Glands- Hillard Flashcards

1
Q

where are the adrenal glands

A

above the kidney in a superior location

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2
Q

what are the parts of the adrenal gland

A

capsule, adrenal cortex, adrenal medulla

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3
Q

what is the blood supply to and from the adrenal glands

A

arteries: suprarenal arteries
vein: adrenal vein

has a robust blood supply so it is less disturbed by interuptions to blood flow

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4
Q

what are the three parts of the adrenal cortex

A

zona glomerulosa

zona fasiculata

zona reticularis

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5
Q

the zona golmerulosa creates _

A

mineralocorticoids: aldosterone which helps to increase salt retention by the kidney

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6
Q

the zona fasciulata creates _

A

cortisol which is released during times of stress

and increases glucose in the blood

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7
Q

the zona reticularis creates

A

androgens

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8
Q

an increase in androgens will have a _ affect

A

virilizing/masculining effect

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9
Q

the adrenal medulla creates _

A

catecholamines (epinephrine and noreepinephirne)

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10
Q

cushing syndrome is caused by an excess of?

A

glucocorticoid cortisol

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11
Q

what are the symptoms of cushing disease

A

HTN- multifactorial cortisol increases vascular sensitivity and drives renin production

fat redistribution- central obesity and fat behind the neck, cheecks, and side of head (moon facies)

secondary diabetes - increased blood sugar levels and increased glucose in the urine

catbolic state breaking down bone and collagen - skin thinning and osteoporosis

mood swinfs

depressed immune system

hirtuism

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12
Q

cortisol is a potent driver of _

A

glucoenogenesis and created glucose from energy stores

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13
Q

high cortisol causes the body to be in a _ (anabolic/catabolic) state

A

catabolic

breakdown bone and collagen

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14
Q

describe the HPA of cortisol

A

increased stress will stimulate CRH release from the hypothalamus, which acts on the the anterior pituitary to release ACTH. ACTH then acts on the adrenal gland (zone fasiculata) to produce cortisol

cortisol has negative feedback on the AP and the hypothalamus

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15
Q

what are the two types of cushing syndrome

A

EXOGENOUS/ IATROGENIC- too much cortisol being absorbed

ENDOGENOUS- body is producing too much cortisol

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16
Q

what is cushing disease

A

problem in the pituitary or higher

usually a pituitary adenoma that causes hyperfunctioning: cortioctrophic adenoma that creates too much ACTH and causes excess cortisol release from the adrenal glands

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17
Q

what is ectopic cushing disease

A

ACTH is produced in an ectopic location most notably due to a paraneoplastic syndrome: small cell carcinoma of the lung

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18
Q

what is an adrenal gland cushing syndrome

A

this is when excess cortisol is being produced from the adrenal glands

most commly from adrenal adenoma, carcinoma or hyperplasia

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19
Q

does adrenal cushing disease dependent on ACTH

A

no, it would actually be suppresed by cortisol negative feedback

cushing disease and ectopic cushing disease are dependent on ACTH - all of them are endogenous

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20
Q

what is the most common cause of ACTH INDEPENDENT endogenous hypercortisolism

A

adrenal adenomas

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21
Q

how does an adrenal adenoma look?

A

well demarcated and yellow cut surface

impossible to tell grossly if it is nonfunctional or secreting cortisol or secreting aldosterone

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22
Q

how do adrenal adenomas look under the microscope?

A

they are well circumscribed tumors

tumors recapitulate cells in the adrenal cortex- can have atypica

  • atypia is not an evidence of malignancy

must look for invasion/metastasis to rule out malignancy

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23
Q

hyperplasia of the adrenal gland is most commonly seen with?

A

pituitary cushing syndrome and paraneoplastic/ectopic cushing syndrome

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24
Q

if hyperplasia of the adrenal gland is the only presenting feature then it may be a result of what genetic disease

A

mcCune albright

or carney complex (PRKAR1A, lactotrophs and somatotrophs)

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25
Q

what is mccune albright syndrome

A

this is replacement of bone with firbous tissue and cafe au lait spots with precoccious puberty

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26
Q

what is carney complex

A

mucocutaneous pigmentation and myocmatous tumors

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27
Q

what does the adrenal gland look like when given exogenous cortisol/corticosteroids

A

atrophic and shrunken

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28
Q

why do the adrenal glands become atrophic and shrunken with chronic corticosteroid use?

A

because they no longer need to produce steroids there will be a loss of cortical layers/cells

must taper off corticosteroids

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29
Q

paraneoplastic cushing syndrome is most often caused by?

A

small cell lung carcinoma

lung is the first source to consider if there is ectopic cushing syndrome

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30
Q

how would you screen someone for cushing syndrome

A

start by taking a good history and physical

make sure they are not taking any exogenous steriods

rule out physiological reasons as to why there is increased cortisol ( pregnancy, obesity)

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31
Q

what are the screening tests for cushing syndrome

A

low dose dexamethasone suppresion test

24 hr urinary cortisol

salivary cortisol (late night)

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32
Q

after the diagnosis of cushings syndrome is made what do we do now?

A

check ACTH levels to see if it is ATCH independent or dependent

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33
Q

if cushing syndrome is ACTH independt ACTH levels will be?

A

suppressed

then you should do imaging of the adrenal glands

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34
Q

if ACTH is dependent in cushing syndrome meaning ACTH levels are?

you then do what?

A

normal to high

high dexamethasone suppresion test
pituitary MRI
inferior petrosal sinus sampling

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35
Q

in a dexmathasone suppresion test what happens

A

in a normal person this should suppress ACTH

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36
Q

in a pituitary adenoma is acth suppresed in a high dexamethasone test?

A

yes

then it is cushing disease!

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37
Q

in ectopic acth tumors does a dexamethasone test suppress ACTH

A

no

so check the lungs for paraneoplastic syndrome: small cell carcinoma of the lungs

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38
Q

what does a pituitary MRI do?

A

it checks for cushing disease (pituitary tumor) by looking at the pituitary

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39
Q

what is an inferior petrosal sinus sampling

A

measuing ACTH directly from the veins rhat drain the pituitary gland to check for tumor

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40
Q

aldosterone is normally released in response to _

A

renin

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41
Q

renin is released by the kidney when?

A

when there is low volume or low blood pressure

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42
Q

renin converts angiotensionogen to

A

angiotensin I

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43
Q

angiotensin I is converted to angiotensin II by?

A

angiotensin converting enzyme (ACE)

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44
Q

angiotension II stimulates the adrenal gland to release _

A

aldosterone

45
Q

what are the actions of aldosterone

A

causes the kidney to absorb sodium and water and increases blood volume

46
Q

what is hyperaldosteronism

A

this is chronic elevation of aldosterone

47
Q

what are the types of hyperalldosteronism

A

primary- adrenal gland produces too much aldosterone and renin is low

secondary- extradrenal cause of too much aldosterone and renin is high

48
Q

explain primary hyperaldosteronism

A

the adrenal gland will produce too much aldosterone which will raise blood pressure and blood volume, this in turn will adequately perfuse the kidney and cause renin to decrease

49
Q

explain secondary hyperaldosteronism

A

decreased blood flow to the kidney (globally or locally) can cause renin to increase and increase aldosterone

50
Q

what is the most common cause of primary hyperaldosteronism

A

idiopathic hyperaldosteronism where there is hyperplasia of the zona glomerulosa

51
Q

what is the second most common type of primary hyperaldosteronism

A

an aldosterone secreting ADENOMA in the zona golmerulosa

aka conn syndtome

52
Q

what is a rare cause of primary hyperaldosteronism

A

glucocorticoid suppresible primary hyperaldosteronism

53
Q

what is glucocorticoid suppresible primary hyperaldosteroinsm

A

hybrid cells ar ein the zona glomerulosa due to a genetic abnormality and release aldosterone in reponse to ACTH

so aldosterone is increased when ACTH increases

54
Q

does the dexamethasone test suppress ACTH in glucocorticoid suppresible primary hyperaldosteronism

A

yes, once ACTH is suppressed then aldosterone release will decrease from teh hybrid cells

55
Q

what are the symptoms of primary hyperaldosteronism

A

hypertension and hypokalemia

hypokalemia can cause a metabolic alkalosis

low postassium- can cause muscle cramps, neuromusclar isssues, weakness

aldosterone increases Na+ absorption and K+ excretion

in practice hypokalemia is inconsistently seen

56
Q

what is the screening test for primary hyperaldosteronism

A

aldosterone to renin ratio

ratio should be high

57
Q

if the aldosterone to renin ratio is high you should do what confirmatory test?

A

a saline infusion (aldosterone suppresion test)

if aldosterone does not suppress you have primary hyperaldosteronism

58
Q

adrenal adenomas in primary hyperaldosteronism can show?- on hsitology

A

spironlactone bodies on histology

used to treat an adrenal adenoma (conns syndrome)

59
Q

what do spironolactone bodies look like?

A

circular whorled eosionophilic structures in the tumor that represent endoplasmic reticulum material bound to aldosterone

60
Q

secondary hyperaldosteronism is caused by conditions that activate?

what are three things that can cause secondary hyperaldosteroinsim

A

activate the RAS system from decreased blood flow to the kidney or from global hypovolemia and edema

renin producing tumor

61
Q

what can lead to decreased renal artery perfusion

A

renal artery stenosis, renal fibromusclar dysplasia

62
Q

what can lead to global hypovolemia and edema

A

heart failure and neprhotic syndrome

63
Q

normal pregnacy can also induce an increase in renin and aldosterone how?

A

due to an increase in ovarian secretion oand decidual release and high estrogen levels which drive production of angiotensionogen

64
Q

congenital adrenal hyperplasia is a group of autosomal _ disorders in wheich the enzyme invovled in the biosynthesis of _ is deficent

A

recessive

cortisol

65
Q

what mutation is involved in CAH and what enzyme is deficient

A

CYP21A2 is mutated

21-hydroylase def

66
Q

congenital adrenal hyperplasia typically presents at _ and shows what sigsn?

A

birth

decreased cortisol
decreased aldosterone
virulization

67
Q

why does adrenal hyperplasia occur in CAH

A

there is decreased cortisol the hypothalamus is no longer inhibited and will create more CRH and the pituitary wont be inhibited from secreting ACTH which will act on the adrenal gland and cause hyperplasia/enarlgement

68
Q

21 hyrdoylase converts precursor products that are originally derived from _ to aldosterone and cortisol

A

cholesterol

69
Q

with a 21 hydroxylase def there is an enzymatic def in the conversion of

A

progesterone becoming 11-dexycorticosterone and eventually aldosterone

17-hydroy progesterone becoming 11-doexycortisol and eventually cortisol

70
Q

because of the 21 hydroylase def in CAH what happens

A

17- hydroxyprogesterone is converted to androsteniodione and eventually androgens like testosterone and give virulizing effects

71
Q

an infant with CAH will look?

A

clitoral enlargment labial fusion in XX

penis enlargement in XY

usually just effects external genitalia

salt wasting

72
Q

low aldosterone leads to

A

salt wasting and hyperkalemia

73
Q

low cortisol leads to?

A

medullary defects, low epinephrine, hypotension and collapse

74
Q

intraadrenal glucocorticoids are necessary for _ production

A

catecholamines (epineprhine and norepinephrine)

75
Q

what test is diagnositc for CAH

A

elevated 17 hydroxyprogesterone (17 OHP_

heel puncture taken 2-4 days after birth

76
Q

what are the three types of CAH

A

classic form with salt wasting- present in neonate

simple virulizing- presents in neonates

non-classic- late onset (present in adolescents/adult)

usually the 21 def is shown on the neonatal newborn screen

77
Q

what is acute primary adrenocorticoal insufficiency

A

there is a problem in the adrenal gland that causes a lack of adrenal hormone from being produced

doesnt make mineralocorticoids or glucorticoids like it should

78
Q

what are the causes of acute primary adrenocortical insuficiency

A

chronic steroid use rapid withdrawl

massive adrenal hemorrhage

waterhouse friederichsen syndrome

79
Q

why does excogenous steroid use and withdrawl cause acute primary adrenocortical insufficiency

A

use of steroids results in atrophy of the adrenal gland, upon removal of them the adrenal gland cannot make the hormones it needs

80
Q

how does acute adrenal hemorrhae cause adrenocortical insufficiency

A

due to the size and vasculatitiry of the adrenal gland at birth any time hypoxia occurs the adrenal gland will recieve more blood and cause increased pressure and hemorrage into the adrenal gland damaging it

81
Q

what is waterhous friederichsen syndrome

A

bilateral adrenal hemorrhage that is a result of neisseria meningitis (infection)

can be causse dby any bacterial organism

82
Q

exogenous steroids can cause _ and rapid withdrawl of steroids can cause

A

cushing syndrome

acute primary adreocortical insufficency

83
Q

neisseria meningitdis is manifested with DIC what is this?

A

intravascular coagulation that causes abnormal clotting through the bodys blood vessels that can ruputre in the adrenal glands

84
Q

key symptoms of primary adrenocaortical insufficiency

A

abdominal pain, nausea, vomiting, weakness, lethary, shock , and confusion

acute adrenocortical insufficiency can also be seen in chronic adrenocortical insuffiency when there is a major stressor

85
Q

what is chronic adrenocortical insufficiency symptoms

A

addisons disease

chronic fatigue !
abdominal pain, N/V,

postural hypotension
hyperpigmentation of the skin

86
Q

why is skin hyperpigmentation seen in addisons disease

A

a feedback loop increases melanin synthesis

low cortisol drives increased production of POMC causing increased ACTH and increased melanocyte stimulating hormone

87
Q

is hyper pigmentation seen in secondary adrenocortical insuficiency

A

no

88
Q

what are the common causes of chronic primary adrenocortical insufficiency

A

autoimmune adrenalitis

infections

metastatic neoplasms

congenital

89
Q

what is autoimmune adrenalitis

what are the mutations

A

the most common cause of primary chronic adrenocortical insuffciency that has a mutation in AIRE genes

APS1- fungal infections, teeth abnormalities, other autoimmune diseases like hypoparathyroidism

APS2- autoummune thyroiditis

90
Q

APS-1 mutations also cause

A

hypoparathyroidism and fungal infections

adrenal isuf. too

91
Q

APS-2 mutations also cause

A

autoimmune thyroiditis

adrenal insuf. too

92
Q

what does autoimmune adrenalitis look like on histology

A

lymphocytic infiltrate actively attacking the adrenal glands

93
Q

what infection can cause primary chronic adrenocortical insufficiency

A

tuberculosis showing caseous necrosis (cheesy) in the adrenal gland

94
Q

congenital adrenal insufficiency can also casue chronic primary adrenocortical insufficency what are the two types

A

adrenoleukodystrophy and adrenal hypoplasia

95
Q

what is adrenoleukodystrophy

A

the accumulationof very long chain fatty acids that cannot be brolen down and settle in the brain, nervous system, and adrenal gland

it is x linked and leads to learning diabilities, sezuires, adrenal insufficiency, and skin hyperpigmentation

96
Q

what is congential adrenal hypoplasia

A

a genetic cause of chronic primary adrenocortical insuffiency that is from an underdeveloped adrenal cortex

97
Q

what is secondary adrenocortical insufficiency

A

adrenal insuffiency due to ACTH secretion def in the pituitary

98
Q

what is tertiary adrenocortical insufficiency

A

low cortisol due to def in CRH secretion

99
Q

what is the major difference in secondary and teritary adrenocortical insufficiency in comparision to primary insufficiency

A

there is no hyperpigmentation

100
Q

adrenal carcinomas are typically _ (small/large) and poorly circumscribed there is necrosis and hemorrhage and _ confirms malignancy

A

large

metastasis or local invasion

poor prognosis -2 years

101
Q

what are adrenal incidentalomas

A

an adrenal mass larger than 1cm that comes to attention on workup

asymptomatic

if they are not causing any problems just repeat imaging

rule out cushing syndrome, pheochromocytoma etc.

any alarming features shuold be worked up

102
Q

what is an adrenal myelolipoma

A

this is a benign lesion that consists of mature fate and hematopoientic elements

trilineage- erythrocytes, myeloid, megakarycytes

grossly: yellow fat with dark hematopoetic areas

103
Q

what is a pheochromocytoma

A

this is a tumor of the adrenal medulla chromaffin cells

it secretes catecholamines

104
Q

what are the sympotms of a pheochromocytoma

A

elevated blood pressure

abrupt elevations in blood pressure with the sudden release of catecholamines

increased HR, sweating, headache, diaphoresis, palpitations, anxiety

105
Q

how do you test for pheochromocytoma?

A

urinary or plasma metanephrines which will be elevated in disease

106
Q

what is the pathophysiology of a pheochrmocytoma

A

release of catecholamines

epinephrine acts on beta 2 receptors and increases cardiac output

norepineprhine works on alpha 1 receptors and increases cardiac output and constricts blood vessels

causes hypertension

107
Q

what is the rule of 10s for a pheochromocytoma

A

10% are extra-adrenal
10% of sporadic tumors are bilateral
10% are malignant
10% do not show hypertension

25% show familal mutations

108
Q

gross apperance of pheochromocytoma

A

well circumscribed, yellow/tan

109
Q

histology of pheochromocytoma

A

discrete nests of supporting sustentacular cells called zellballen

endoctine atypia (doesnt mean malignancy)

ENRP Exam 2 (13 decks)

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