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Endocrine II midterm > Guerin: Adrenal Gland > Flashcards

Guerin: Adrenal Gland Flashcards

1
Q

Where is cortisol made?

A

Zona fasciculata

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2
Q

Where is aldosterone made?

A

zona glomerulosa

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3
Q

Where are the sex steroids made?

A

Zona reticularis

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4
Q

What does the adrenal medulla make?

A

-catechohlamines like epinephrine

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5
Q

What does the 3 zonas (GFR) make up?

A

The adrenal cortex

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6
Q

Cushing syndrome

A

-excess cortisol

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7
Q

most common cause of cushing syndrome

A

-exogenous glucocorticoids

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8
Q

What is it called when we have excess cortisol due to too much ACTH?

A
  • Cushing DISEASE

- pituitary probs (adenoma)

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9
Q

When do we most often see secretion of ectopic ACTH by nonpituitary tumors?

A

-small-cell carcinoma of the lung

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10
Q

So, what causes the ACTH-independent Cushing syndrome then?

A
  • primary adrenal neoplasms (most commonly)
  • adrenal adenoma (10%)
  • adrenal carcinoma (5%): produce most profound hypercortisolism
  • high cortisol, low ACTH
  • primary cortical hyperplasia ia uncommon… vast majority of hyperplastic adrenals are ACTH dependent
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11
Q

What will we see in the adrenal glands if there is exogenous glucocorticoids?

A
  • cortical atrophy

- suppression of ACTH… lack of stimulation of the zonae fasciculata and reticularis

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12
Q

When do we see diffuse hyperplasia (both glands enlarged)?

A
  • endogenous hypercortisolism
  • ACTH-dependent Cushing syndrome
  • Cortex can be variably nodular
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13
Q

Macronodular hyperplasia

A
  • endogenous hypercortisolism
  • adrenals almost entirely replaced by prominent nodules of varying sizes
  • areas between the macroscopic nodules also demonstrate evidence of microscopic nodularity
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14
Q

Micronodular hyperplasia

A
  • endogenous hypercortisolism
  • composed of 1-3 mm darkly pigmented (brown to black) micronudles, with atrophic intervening areas
  • Pigment is through to be lipofuscin
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15
Q

What do adrenocortical adenomas look like?

A
  • benign
  • yellow tumors surrounded by thin or well-developed capsules
  • most weigh <30 gm
  • Microscopically see cells that look like normal zona fasciculata
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16
Q

What do adrenocortical carcinomas look like?

A
  • LARGER than the adenomas (200-300 gm)… remember… size matters!
  • Unencapsulated
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17
Q

Clinical course of adrenal neoplasms

A
  • symptoms develop slowly over time
  • early: htn and weight gain
  • later more characteristic features: central pattern of fat deposition, moon facies, fat in the posterior neck and back
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18
Q

What happens to the fast twitch fibers in adrenal neoplasms?

A
  • atrophy

- decreased muscle mass and proximal limb weakness

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19
Q

What happens because of the increased glucocorticoids from the adrenal neoplasm?

A
  • hyperglycemia

- secondary diabetes

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20
Q

What are the catabolic effects of an adrenal neoplasm?

A
  • loss of collagen and resorption of bones
  • skin is thin, fragile, and easily bruised, poor wound healing, cutaneous striae are particularly common in abdominal area, osteoporosis
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21
Q

Why is there an increased risk of infection with adrenal neoplasms?

A

-you have immune suppression from all of the glucocorticoids probably

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22
Q

Diagnosis of Cushing syndrome

A
  • increased 24 hour urine free-cortisol

- loss of normal diurnal pattern of cortisol secretion

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23
Q

how do we determine the cause of cushing syndrome?

A
  • serum ACTH

- dexamethasone suppression test: urinary excretion of 17-hydroxycorticosteroids after administration of dexamethasone

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24
Q

out the options of pituitary cushing, ectopic ACTH, and Adrenal tumor, which of those is the only one that shows suppression from a high does of dexamethasone?

A

-Pituitary cushing

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25
Q

What is primary hyperaldosteronism?

A
  • autonomous overproduction of aldosterone
  • suppression of the renin-angiotensin system and decreased plasma renin activity
  • elevated BP is the most common manifestation
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26
Q

Whatare the 3 mechanisms that cause primary hyperaldosteronism?

A
  • adrenocortical neoplasm
  • bilateral idiopathic hyperaldosteronism (IHA)
  • Glucocorticoid-remediable hyperaldosteronism
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27
Q

What is an adrenal adenoma called?

A

Conn syndrome

  • middle age most common
  • rarely carcinoma
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28
Q

Which side do adenomas usually show up on?

A

-left

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29
Q

What is the characteristic microscopic feature of adenomas?

A

-spironolactone bodies

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30
Q

What are spironolactone bodies?

A
  • eosinophilic, laminated cytoplasmic inclusions

- found after treatment with spironolactone (antihypertensive drug)

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31
Q

Will an adrenal adenoma suppress ACTH secretion?

A

no

-so adjacent adrenal cortex and contralateral gland are not atrophic

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32
Q

Bilateral idiopathic hyperaldosteronism

A
  • most common cause of primary hyperaldosteronism
  • pts older
  • less severe hypertension
  • pathogenesis is unclear
  • bilateral nodular hyperplasia of the adrenal glands
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33
Q

Glucocorticoid-remediable hyperaldosteronism

A
  • uncommon
  • familial hyperaldosteronism
  • rearrangement of chromosome 8 that places the gene for aldosterone synthase under the control of the ACTH responsive gene promoter
  • ACTH stimulates the production of aldosterone
  • suppressible by dexamethasone!
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34
Q

Secondary Hyperaldosteronism

A

-sldosterone released in response to activation of the renin-angiotensin system by increased plasma renin

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35
Q

When do we see secondary hyperaldosteronism?

A
  • decreased renal perfusion
  • arterial hypovolemia and edema
  • pregnancy (estrogen increases renin…increased aldosterone)
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36
Q

What is the most important clinical thing with secondary hyperaldosteronism?

A
  • Hypertension!!!
  • aldosterone promotes sodium reabsorption… increased water reabsorption too
  • K+ wasting
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37
Q

Diagnosing secondary hyperaldosteronism?

A

-elevated ratio of plasma aldosterone concentration to plasma renin activity

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38
Q

Confirmation test for secondary hyperaldosteronism

A
  • aldosterone suppression test
  • administer oral saline load, IV saline load, fludrocortisone
  • If aldosterone is not suppressed: confirm primary hyperaldosteronism*
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39
Q

Tx of hyperaldosteronism

A
  • varies depending on cause
  • adenomas: surgical resection
  • Bilateral hyperplasia: medication… aldosterone antagonist (Spironolactone)
  • secondary hyperaldosteronism: tx underlying cause
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40
Q

CAH

A
  • congenital adrenal hyperplasia
  • auto recessive inherited metabolic errors
  • enzyme probs
  • if lots of androgens…. virilization
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41
Q

21 hydroxylase deficiency

A
  • most common one by far

- Mutations of CYP21A2

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42
Q

What three distincitve syndromes come with 21 deficiency?

A
  • Salt-wasting syndrome
  • simple virilizing adrenogenital syndrome without salt wasting
  • nonclassic or late-onset adrenal virilism
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43
Q

Salt wasting syndrome

A
  • inability to convert progesterone into Deoxycorticosterone
  • typically presents soon after birth
  • salt wasting (hyponatremia and hyperkalemia)…. acidosis, htn, CV collapse, and possibly death
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44
Q

Simple virilizing adrenogenital syndrome without salt wasting

A
  • presents as genital ambiguity
  • 1/3 of 21 hydroxylase deficiency
  • progressive virilization
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45
Q

Nonclassic or late-onset adrenal virilism

A
  • most common
  • only a partial deficiency in 21 hydroxylase function
  • asymptomatic
  • hirsutism, acne, and menstrual irregularities
46
Q

Morphology of CAH

A
  • adrenals are bilaterally hyperplastic
  • cortex is thickened and nodular
  • cortex looks brown… due to total depletion of all lipid
47
Q

Clinical course of CAH

A
  • androgen excess, WITH OR WITHOUT aldosterone and glucocorticoid deficiency
  • onset of symptoms can be perinatal period, later childhood, or adulthood
48
Q

What does 21 deficiency look like in females?

A
  • clitoral hypertrophy and psuedohermaphroditism in infants

- oligomenorrhea, hirsutism, and acne in postpubertal

49
Q

What does 21 deficiency look like in males?

A
  • enlagement of the external genitalia and other evidence of precocious puberty in prepubertal patients
  • oligospermia in older males
50
Q

What should be suspected in any neonate witha mbiguous genitalia?

A

-CAH!

51
Q

CAH effects on adrenal medulla

A
  • high levels of intra-adrenal glucocorticoids are required to make catecholamine
  • pts with severe salt-wasting 21 hydroxylase deficiency: low cortisol levels… adrenomedullary dysplasia…. hypotension and circulatory collapse
52
Q

Tx of CAH

A
  • exogenous glucocorticoids….. suppress ACTH levels

- mineralocorticoid supplementation is required in the salt-wasting variants of CAH

53
Q

What did JFK have and what is it?

A

Addison disease

-primary Adrenocortical insufficiency

54
Q

What is adrenal crisis?

A

Primary acute adrenocortical insufficiency

55
Q

Primary acute adrenocortical insufficiency… what was bolded on that slide?

A

-Exogneous corticosteroids, in whom rapid withdrawal of steroids or failure to increase steroid doses in response to an acute stress

56
Q

What is something involving blood that can cause primary acute adrenocortical insufficiency?

A
  • Massive adrenal hemorrhage… damage to the adrenal cortex
  • Newborns following a difficult delivery
  • Pts on anticoagulant therapy
  • Postsurgical pts who develop DIC
  • Disseminated bacterial infection (Waterhouse-Friderichsen syndrome)
57
Q

Waterhouse-Friderichsen Syndrome

A
  • uncommon
  • overwhelming bacterial infection…. classically Neisseria meningitidis septicemia
  • rapidly progressive hypotension leading to shock
  • disseminated intravascular coagulation associated with widespread purpura, particularly of the skin
  • rapidy developing adrenocortical insufficiency associated with massive BILATERAL adrenal hemorrhage
  • fatal, unless promptly recognized and treated
58
Q

Primary chronic adrenocortical insufficiency

A
  • ADDISON DISEASE!
  • uncommon
  • progressive destruction of the adrenal cortex
  • takes ~90% of cortex loss to get symptoms
59
Q

Autoimmune Adrenalitis

A
  • accounts for 60-70% of addison disease
  • Autoimmune destruction of Steroidogenic cells
  • Autoantibodies to 21 hydroxylase and 17 hydroxylase have been detected
60
Q

What two clinical settings does Autoimmune Adrenalitis occur in?

A
  • Autoimmune polyendocrine syndrome type 1 (APS1)

- APS2

61
Q

APS1

A
  • AKA Autoimmune polyendocrinopathy, candidiasis, and ectodermal dystrophy (APECED)
  • chronic mucocutaneous candidiasis
  • abnormalities of skin, dental enamel, and nails
  • lots of AI disorders
  • Mutatioins in the Autoimmune regulator (AIRE) gene on chromosome 21q22
62
Q

What is AIRE used for?

A
  • Central T-cell tolerance to peripheral tissue antigens is compromised…. autoimmunity
  • Also develop autoantibodies against IL-17 and 22 (Crucial for defense against fungal infections)… that is for APS1
63
Q

APS2

A
  • usually starts in early adulthood

- presents as a combo of adrenal insufficiency and autoimmune thyroiditis or type 1 diabetes

64
Q

In regards to metastatic neoplasms, which of them contributes to Addison disease?

A

-lung and breast carniomas most commonly

65
Q

What are some genetic causes of Addison disease?

A
  • congenital adrenal hypoplasia… rare x-linked disease

- Adrenoleukodystrophyq

66
Q

What infections can cause Addison disease?

A
  • TB and fungal kinds

- AIDS at increased risk from several infeectious and noninfectious causes

67
Q

Morphology of primary autoimmune adrenalitis

A
  • irregularly shrunken glands, which may be difficut to identify grossly
  • histologically: cortex contains scattered residual cortical cells in a collapsed netwrok of connective tissue, variable lymphoid infiltrate
68
Q

Morphology of tuberculous and fungal disease (As a cause of Addison)

A

-adrenal architecture is effaced by a granulomatous inflammatory reaction

69
Q

Morphology of metastatic carcinoma as a cause of addison disease?

A

-normal architecture obscured by the infiltrating neoplasm

70
Q

Clinical course of addison disease

A
  • begins insidiously
  • early manifestations: progressive weakness and easy fatigability: often dismissed as nonspecific complaints
  • GI disturbances: anorexia, nausea, vomiting, weight loss, and diarrhea
  • Primary adrenal disease: get hyperpigmentation of the skin… results from elevated levels of POMC
71
Q

Why do we have elevated levels of POMC?

A

-comes from ant pit and ais a precursor of both ACTH and MSH

72
Q

In addison disease, will there by hyper or hypoglycemia?

A
  • Hypoglycemia can occur

- from impaired gluconeogenesis

73
Q

What else can precipitate an acute adrenal crisis?

A
  • Stresses!
  • infections, trauma, prodecures
  • intractable vomiting, abdominal pain, hypotension, coma, and vascular collapse
  • death occurs rapidly unless corticosteroid therapy begins immediately
74
Q

Secondary adrenocortical insufficiency

A
  • any disorder of the hypothalamus and pituitary that reduces the output of ACTH
  • Prolonged exogenous glucocorticoids… suppressed ACTH and adrenal function
  • Dont see hyperpigmentation of primary addison
  • Low cortisol and androgen output but normal or near-normal aldosterone… don’t see hyponatremia and hyperkalemia
  • Exogenous ACTH….. prompt rise in plasma cortisol levels
75
Q

Adrenocortical Neoplasms

A
  • majority are sporadic

- Two familial cancer syndromes with increased risk of adrenocortical carcinomas

76
Q

What are those two familial cancer syndromes with increased risk of adrenocortical carcinomas?

A
  • -Li-Fraumeni syndrome: germline TP53 mutations

- Beckwith-Wiedemann Syndrome

77
Q

If we have hyperaldosteronism and cushing syndrome, what is that most likely from?

A

-a functional adenoma

78
Q

If we have a virilizing neoplasm, what is that most likely from?

A

-carcinoma

79
Q

Adrenocortical adenomas

A
  • most are clinically silent and discovered incidientally
  • well-circumscribed nodular lesion
  • usually yellow to yellow-brown because of the presence of lipid
  • cortex adjacent to nonfunctional adenomas is normal
  • if it is functional, the cortex is usually atrophic
80
Q

Adrenocortical carcinomas

A
  • Rare
  • functional: virilism
  • large, invasive
  • invade adrenal vein, vena cava, and lymphatics
  • Median pt survival is 2 years
81
Q

What does adrenocortical carcinoma look like microscopically?

A
  • looks like an adenoma (Pretty bland)

- Undiferentiated carcinoma

82
Q

What is something that is much more common that we will need to separate from undifferentiated carcinomas of the adrenal cortex?

A
  • metastases to the adrenal cortex

- much more common

83
Q

Adrenal myelolipomas

A

-benign lesions composed fo mature fat andhematopoietic cells

84
Q

Adrenal incidentaloma

A
  • frequent abdominal imaging led to discovery many incidental adrenal masses
  • asymptomatic
  • small nonsecreting cortical adenomas
85
Q

Adrenal Medulla, what’s it made of?

A
  • chromaffin cells (specialized neural crest cells)

- Supporting (sustentacular) cells

86
Q

What comes from the adrenal medulla?

A

-catecholamines (E and NE)

87
Q

What is the paraganglion system composed of?

A

adrenal medulla and extra-adrenal paraganglia (made up of chromaffin cells)

88
Q

What are the most important neoplasms of the adrenal medulla?

A
  • Pheochromocytomas

- Neuroblastomas and other neuroblastic tumors

89
Q

Pheochromocytomas

A
  • neoplasms composed of chromaffin cells
  • make and release catecholamines and sometimes peptide hormones
  • rare cause of surgically correctable htn
90
Q

What is the Rule of 10’s?

A
  • for pheochromocytomas
  • 10% are extra-adrenal (paragangliomas)
  • 10% of sporadic pheochromocytomas are bilateral
  • 10% are malignant (more common in extra-adrenal paragangliomas
  • 10% don’t have htn
91
Q

Paroxysmal episodes with Pheochromocytomas

A
  • Abrupt, precipitous elevation in blood pressure
  • caused by sudden release of catecholamines
  • Tachycardia, palpitations, headache,.. etc…
92
Q

Familial pheochromocytomas

A
  • up to 25 % of pts with pheochromocytomas and paragangliomas
  • typically younger
  • more often bilateral… up to 50%
93
Q

What gene is wrong with MEN2A and b

A

RET

  • 2A: Pheo, Medullary thyroid carcinoma, pt hyperplasia
  • 2B: Pheo, Medullary thyroid carcinoma, marfanoid habitus, mucocutaneous GNs
94
Q

Morphology of Pheochromocytomas

A
  • small to large…. wide range
  • histology is variable
  • very difficult to determine if pheochromocytomas are malignant
95
Q

How is the malignancy of a pheochromocytoma defined?

A

-by the presence of metastases

96
Q

Dx pheochromocytoma

A
  • Increased urinary excretion of free catecholamines and metabolites
  • VMA and metanephrines
97
Q

Tx of Pheochromocytoma

A
  • for isolated benign tumors: surgical excision
  • OMG TX WITH ADRENERGIC-BLOCKING AGENTS PREOPERATIVELY AND INTRAOPERATIVELY TO PREVENT A HYPERTENSIVE CRISIS
  • For multifocal lesions: medical treatment for htn
98
Q

Multiple Endocrine Neoplasia (MEN) syndromes

A
  • younger age than sporadic tumors
  • multiple endocrine organs
  • often multifocal in an effected organ
  • usually preceded by an asymptomatic stage of hyperplasia involving the cell of origin
  • tumors are usually preceded by an asymptomatic stage of hyperplasia involving the cell of origin
  • tumors are usually more aggressive
99
Q

MEN1

A
  • Wermer’s syndrome

- 3 P’s: pituitary adenoma, parathyroid tumors, pancreatic tumors

100
Q

MEN2A

A
  • Sipple syndrome
  • Hyperparathyroidism
  • medullary thyroid cancer
  • pheochromocytoma
101
Q

MEN2B

A
  • Marfanoid phenotype
  • Mucosal neuromas
  • Pheochromocytomas
  • Ganglioneuromas
102
Q

what kind of pituitary adenoma will we most likely see in MEN1?

A

-prolactinoma

103
Q

What is the leading cause of morbidity and mortality in MEN1?

A

-Pancreas endocrine tumors

104
Q

where is the most common site of gastrinomas (MEN1)

A

-duodenum

105
Q

What is the MEN1 mutation

A

-germline mutations in the MEN1 tumor suppressor gene

106
Q

MEN2A

A
  • Sipple syndrome
  • Pheochromocytoma
  • Medullary thyroid carcinoma (100%*)
  • Parathyroid hyperplasia
107
Q

What mutation happens with MEN2A?

A

-germline mutations in RET proto-oncogene

108
Q

MEN2B

A
  • Medullary Carcinoma of the thyroid (100%)
  • Pheochromocytomas
  • Neuromas or ganglioneuromas of the skin, oral mucosa, eyes, resp tract, and GI tract
  • Marfanoid habitus
  • different RET mutation than MEN1
109
Q

Which MEN is the aggressive diseases and has an adverse prognosis?

A

-MEN2B

110
Q

What is recommended for family members of ppl with MEN2B?

A
  • Genetic screening
  • offered prophylactic thyroidectomy to prevent medullary thyroid carcinoma
  • life-threatening
111
Q

Familial medullary thyroid cancer

A
  • Variant of MEN-2A
  • only medullary thyroid cancer
  • pts older
  • more indolent course
112
Q

Pineal gland

A
  • minute, pinecone-shaped
  • composed of pineocytes: photosensory and neuroendocrine functions
  • secretes melatonin: control of circadian rhythms
  • Tumors are rare

Endocrine II midterm (13 decks)

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