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Endocrine II - Exam 1 > Adrenal Pathology - MT > Flashcards

Adrenal Pathology - MT Flashcards

1
Q

What are the zones of an adrenal gland and what do they produce respectively?

A

capsule*

  • outermost zona glomerulosa: mineralcorticoids (aldosterone
  • zona fasciculata: glucocorticoids (cortisol)
  • zona reticularis: glucocorticoids (cortisol), sex steroids (estrogen, androgens)
  • Innermost adrenal medulla: chromaffin cells (catecholamines: Epi, NE)
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2
Q

what are 3 syndromes of adrenal hyperfunction? (hyperadrenlism) and what are they characterized by?

A
  • Cushing syndrome: excess cortisol
  • Hyperaldosteronism: excess aldosterone
  • Adrenogenital or virilizing syndromes: excess androgens
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3
Q

What might you see on physical examination of a patient with cushing syndrome?

A
  • central obesity
  • moon facies
  • abdominal striae
  • HTN
  • abnormal hair growth
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4
Q

What can cause exogenous Cushing syndrome?

A
  • Elevated glucocorticoid levels (cortisol) from steroid use (iatrogenic)
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5
Q

What conditions can cause ACTH dependent endogenous cushing syndrome?

Which is most likely in most cases to be the cause (70%)?

A
  • ACTH secreting pituitary adenomas (cushing disease)
  • Secretion of ectopic ACTH by nonppituitary tumor (small cell carcinoma of lung)
  • most likely ACTH sec pituitary adenoma (70%)
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6
Q

What can cause ACTH independent endogenous cushing syndrome?

What lab values indicate these conditions?

A
  • Adrenal adenoma (primary adrenal neoplasms) (10%)
  • Adrenal carcinoma (5%)
  • elevated serum cortisol, low ACTH
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7
Q

Crooke hyaline change, what is it?

A
  • most common alteration resulting from high levels of endogenus or exogenous glucocorticoids d/t accumulation of intermediate keratin filaments in cytoplasm (homogeneous and pale CTH producing cells in anterior pituitary)
  • slide 13 visual
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8
Q

Abnormalities found in Hypercortisolism (Cushing syndrome)

A
  • Cortical atrophy
  • Diffuse hyperplasia
  • Macronodular or Micronodular hyperplasia
  • Adenoma or carcinoma
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9
Q

visuals on diffuse hyperplasia of adrenal gland and also micronodular adrenocortical hyperplasia visual

A

slide 15 and 16

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10
Q

Clinical course of cushing syndrome

  • presentation in early stages
  • later presentation
A
  • HTN and weight gain
  • Truncal obesity, moon facies, accumulation of fat in posterior neck and back (buffalo hump).
  • decreased muscle mass and proximal limb weakness d/t selective atrophy of fast twitch type 2 myofibers
  • hyperglycemia, glucosuria, polydipsia (2nd diabetes)
  • loss of collagen skin thin fragile, easy bruising
  • STRIAE in abdominal area
  • Osteoporosis resultant from bone resorption
  • Mental disturbancs
  • HIrutism, menstrual abnormalities
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11
Q

What lab can be useful in the diagnosis of Cushing syndrome?

A
  • 24 hour urine free cortisol concentration (increased in cushings)
  • DEXAMETHASONE suppression test
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12
Q

What is involved in a dexamethasone suppression test?

A
  • in Pituitary cushing disease (most common form), ACTH levels that are elevated are not suppressed by admin of low dose dexamethasone –> no reduction in urinary excretion of 17 hydroxycorticosteroids

* increased dexamethasone will reduce ACTH secretion–>reduced urine steroid secretino

  • Ectopic ACTH secretion causing ACTH elevation will have complete insensitivity to low or high dose of exogenous dexamethasone
  • Adrenal tumor cushing syndrome ACTH low and will not respond to dexamethasone
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13
Q

Cushing syndrome workup steps

A
  • 24 hour urine cortisol

* if high (>100 mg/dL) suspect cushing problem (syndrome, disease, ectopic)

  • Check ACTH levels

* low, suspect cushing syndrome–>CT/MRI adrenals

* high, suspect disease/ectopic–> high dose dexamethasone test

  • if suppressed, investigate cushing disease

* MRI pituitary (mass ACTH producing, no mass = inf petrosal sinus sampling)

  • if not suppressed, investigate ectopic ACTH w/ CT of chest
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14
Q

cushing disease affects what populatin more?

what is causing it in most cases?

A

Young adult women

* most cases it is an ACTH producing pituitary microadenoma (endogenous ACTH dependent)

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15
Q

The most common cause of hypercortisolism (cushing syndrome) is?

A

Exogenous administration of steroids

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16
Q

How does primary hyperaldosteronism arise?

What conditions can cause primary Hyperaldosteronism?

A
  • Primary

autonomous overproduction of aldosterone, w/ suppression of renin angiotensin system and DECREASED plasma renin activity

* Blood pressure elevation most common manifestation

  • B/l idiopathic hyperadosteronism (IHA)
  • Adrenocortical neoplasms
  • Glucocorticoid remediable hyperaldosteronism

-

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17
Q

What type of condition causing primary hyperaldosteronism is characterized by b/l nodular hyperplasia of adrenal glands and is the most common underlying cause of primary hyperaldosteronism? (60%)

  • what population does this condition tend to affect?
A

B/l idiopathic hyperaldosteronism (IHA)

older people with less severe HTN than those presenting with adrenal neoplasms

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18
Q

What causes Conn syndrome?

What population does it affect?

A
  • Solitary aldosterone secreting adenoma (35% of primary hyperaldosternism cases) (adrenocortical neoplasm)
  • adult middle life more common in women 2:1
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19
Q

What is the genetic rearrangement that can cause Glucocorticoid remediable hyperaldosteronism? (Familial primary hyperaldosteronism)

what does this gene encode?

A

CYP11B2 gene which encodes aldosterone synthase

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20
Q

What is the physiolgy that leads to secondary hyperaldosteronism?

Condition that cause this?

A

Aldosterone release occurs in response to activatino of renin angiotensin system

NON adrenal causes…

  • Decreased renal perfusion (arteriolar nephrosclerosis, renal artery stenosis)
  • Arterial hypovolemia and edema (CHF, cirrhois, nephrotic syndrome)
  • Pregnancy (d/t estrogen induced increases in plasma renin substrate)
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21
Q

What strucure might you see on histology of an aldosterone producing adenoma?

A

Spironolactone bodies (slide 28)

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22
Q
  • most important clinical consequence of hyperaldosteronism is?
A

HTN

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23
Q

Long term effects of hyperaldosteronism induced HTN are?

A

Cardiovascular effects like:

  • Left ventricular hypertrophy
  • reduced diastolic volumes

increase incidence of strokes and MIs

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24
Q

diagnosis of primary hyperaldosteronism is confirmed by what?

if this test is positive, what do you confirm it with?

A

elevated ratios of plasma aldosterone concentration to plasma renin activity

(elevated Plasma aldosterone / Plasma renin activity)

  • if screening is positive, confirm with aldosterone suppression test
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25
Q

What are adrenogenital syndromes?

What causes them?

A

Disorders of sexual differentiation, such as virilization or feminization.

Caused by primary gonadal disorders and several primary adrenal disorders

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26
Q

Adrenal cortex secretes what two compounds that can be converted to testosterone in peripheral tissues?

A

dehydroepiandrosterone and androstenedione

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27
Q

What disorders can cause androgen excess?

A

Adrenocortical neoplasms, and

Congenital adrenal hyperplasias (CAH)

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28
Q

What is Congenital adrenal hyperplasia?

A

Several autosomal recessive inherited metabolic errors characterized by deficiency or total lack of a particular enzyme involve in biosynth of cortical steroids, particularly cortisol

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29
Q

Most common enzyme deficient in CAH?

A

21 hydroxylase deficiency 90% cases (mutation of CYP21A2)

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30
Q

3 distinct syndromes described for CAH?

A
  1. Salt wasting (classic) adrenogenitalism
  2. Virilizing adrenogenitalism (simple)
  3. Non classic adrenogenitalism
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31
Q

In CAH the salt wasting syndrome results from what?

  • how does it cause salt wasting?
A
  • Lack of Hydroxylase (inability to convert progesterone into deoxycorticosterone)
  • results in vitually no synthesis of mineralcorticoids
  • also concomitant block in conversion of hydroxyprogesteroneinto deoxycortisol resulting in deficient cortisol synthesis
32
Q

Pattern of salt wasting is usually detected when?

Why is it detected then and not in utero?

A
  • after birth, becuase in utero maternal kidneys can maintain electrolytes and fluid for baby
  • salt wasting, leads to hyponatremia, hyperkalemia–> acidosis, hypotension–>death from CV collapse
33
Q

What causes the virilization in CAH?

A
  • The block in cortisol production and resultant excess production of androgens

*recognized sooner in females at birth or in utero, males later b/c salt losing crisis

34
Q

What is simple virilizing adrenogenital syndrome?

A

occurs in 1/3 patients with 21 hydroxylase deficiency

  • no salt wasting, sufficient mineralcorticoid production
  • lowered glucocorticoid level fails to cause feedback inhibition of ACTH secretion–>increased testosterone w/ resultant progressive virilization
35
Q

What is late onset non classic adrenal virilism?

A

partial deficiency in 21 hydroxylase, thus late onset

more common than simple virilizing adrenogenital sydrome

36
Q

Clinical sx of late onset adrenal virilism?

A
  • mild manifestations of Hirsutism, acne, irregular menses

*cannot be diagnosed on routine newborne screening

37
Q

Clinical features of CAH are determined by what?

A

specific enzyme deficiencies and include abnormalities related to androgen excess w/ or w/o galdosterone and glucocorticoid deficiency

38
Q

21 hydroxylase deficiency excessive androgenic activity causes what?

A

masculinization in females

ranging effects of:

  • clitoral hypertrophy
  • pseudohermaphroditism in infants
  • oligomenorrhea, hirsutism, acne in post pubertal females
39
Q

Androgen excess associated with what in males?

A

Large penises

precocious puberty in prepubertal patients

oligospermia in older males

40
Q

When should CAH be suspected in neonates?

A

in any neonate with ambiguous genitalia

41
Q

3 causes of adrenocortical insufficiency

A
  • Primary acute adrenocortical insufficiency (adrenal crisis)
  • Primary chronic adrenocortical insufficiency (Addison disease)
  • secondary adrenocortical insufficiency
42
Q

Primary acute adrenocortical insufficiency causes… (x4)

A
  1. Crisis from any form of stress in chronic adrenocortical insufficiency individuals
  2. patients maintained on exogenous steroid who have a rapid withdrawal of those steroids
  3. Massive adrenal hemorrhage (post partum in a difficult delivery for baby where there is trauma and hypoxia)
  4. anticoagulant therapy patients, DIC patients, Waterhous friderichsen syndrome
43
Q

What causes Waterhouse-Friderichsen syndrome?

what characterizes this condition?

A

overwhelming bacterial infection from Neisseria meningitidis septicemia

  • ocassional from Pseudomonas, Haemophilus, Staphylococci
  • rapid progressive hypotension leading to shock
  • DIC associated w/ purpura widespread
  • massive b/l adrenal hemorrhage
44
Q

What causes primary chronic adrenocortical insufficiency? (Addison disease)

A
  • Autoimmune adrenalitis (60-70% most common)
  • Infections (TB, fungi)
  • Metastatic neoplasms (carcinomas of lung and breast)
45
Q

What are the main causes of autoimmune adrenalitis in Addison disease?

A

Autoimmune polyendocrine syndrome type I (APS 1)

type 2 (APS2)

46
Q

Clinical symptoms of Addison’s disease?

A
  • insidious onset
  • progressive weakness nd easy fatigability
  • GI distrubances: anorexia, nausea, vomit, weight loss, diarrhea
  • hyperpigmentation of skin caused by elevated POMCs derived from anterior pituitary
  • Hypotension (Hyperkalemia, hyponatremia, volume depletion)
47
Q

Secondary Adrenocortical insufficiency definition?

A

any disorder of Hypothalmus and Pituitary that reduces output of ACTH –> hypoadrenalism similar to addison disease

48
Q

How do you differentiate secondary adernocortical insufficiency from primary addison disease?

A
  • Lack of hyperpigmentation found in addison disease b/c levels of melanocyte stim hormone are not elevated
  • Secondary hypoadrenalism characterized by deficient cortisol and androgen output but NORMAL aldosterone synthesis

(no hyponatremia and hyperkalemia seen!!!)

49
Q

What adrenocrtical neoplasm are common in adults?

Children?

A
  • Adenomas and Carcinomas equal in adults
  • Carcinomas in children predominate
50
Q

What are 2 familial cancers syndromes associated w/ a predisposition for developing adrenocortical carcinomas?

What is the root cause of each?

A
  • Li- Fraumeni syndrme (TP53 germline mutation)
  • Beckwith-Wiedemann syndrome (epigenetic imprinting)
51
Q

Functional Adenomas are most commonly associated with what?

Virilizing neoplasms associated with what?

A
  • Hyperaldosteronism and Cushing syndrome
  • Carcinoma
52
Q

how is an adrenal cortical adenoma distinguished from nodular hyperplasia?

A
  • solitary circuscribed nature
  • functinoal status (cannot be predicted from microscopic appearance)
53
Q

A histological feature of adrenal cortical adenoma is

A
  • vacuolated neoplastic cell d/t intracytoplasmic lipid
54
Q

Adrenalcortical carcinomas:

  • population affected?
  • functional or non functional?
  • Associated with what?
  • Most appear as
A
  • any age, childhood too
  • functional (more so than adenomas)
  • Virilism or other clinical manifest of hyperadrenalism
  • Large invasive lesion
55
Q

Carcinomas of what origin may likely metastasize to adrenals?

  • likelihood of metastases in adrenals vs. primary adrenocortical carcinoma?
A
  • Bronchogenic origin
  • metastases into adrenals more common than primary adrenocortical carcinomas
56
Q

The vast majority of adrenal incidentalomas prognostically are?

A
  • small nonsecreting cortical adenomas of no clinical importance. In asymptomatic individuals
57
Q

Gross inspection of an adrenal carcinoma is likened to what?

A
  • hamburger meat (caused by hemorrhagic and necrotic tumor)
58
Q

Nml adrenal vs Adrenal carcinoma histology

A

slide 59

59
Q

Cellular composition of the adrenal medulla consists of?

A
  • Chromaffin cells ( specialized neural crest cells aka neuroendocrine cells)
  • Supporting Sustentacular cells
60
Q

Adrenal medulla provides a major source of what for the body?

A
  • Catecholamines (Epi, NE)
61
Q
  • Neuroendocrine cells similar to chromaffin cells are widely dispersed where?
  • What composes the paraganglion system?
A
  • An extra adrenal system of clusters and nodules
  • Extra adrenal system of clusters and nodules + Adrenal medulla

(branchiomeric, Intravagal, Aoticosympathetic)

62
Q

The most important disease of adrenal medulla are?

A

Neoplasms:

  • Pheochromocytoma (neoplasm of chromaffin cells)
  • Neuroblastic tumors (neuronal neoplasms)
63
Q

CT scan of a pheochromocytoma

A

slide 62

64
Q

Why is it important to recognize and dx Pheochromocytomas?

A
  • Rare cause of surgically correctable HTN
65
Q

What can pheochromocytomas produce?

A
  • Catecholamines
  • peptide hormones in some instances
66
Q

Rule of 10s for Pheochromocytomas?

A
  • 10% are extra adrenal @ sites like organs of zuckerkandl and carotid body (Paragangliomas)
  • 10% sporadic adrenal type are bilateral (can be 50% in familial)
  • 10% adrenal type biologically malignant (more common in Paragangliomas) (germline mutation)
  • 10% adrenal type not associated with HTN

if HTN present, 2/3 paroxysmal epiodes

67
Q

25% of individuals with pheochromocytomas and paragangliomas harbor what?

A

Germline mutation

Pheochromocytoma (RET, NF1)

Paraganglioma (SDHC)

68
Q

Gross observation of a pheochromocytoma would show?

A
  • tumor enclosed in an attenuated cortex w/ areas of hemorrhage
69
Q

Histologic change in Pheochromocytomas

A
  • nest of cells (zellballen)
  • salt and pepper chromatin
70
Q

recognize dense core granules on EM for Pheochromocytoma

A

slide 68

71
Q

Clinical presentation of Pheochromocytoma

A
  • dominant manifestation is HTN in 90% of patients
  • 2/3 of patients presenting with HTN will have episodic paroxysmal HTN
  • episodes associated with tachycardia, palpitations, HA, sweating, tremor, sense of apprehension
72
Q

What can precipitate paroxysmal episodes of HTN in patients with pheochromocytomas?

A
  • emotional stress, exercies, change in posture, palpation in region of tumor
73
Q

Acute consequences of suddne catecholamine release in a paroxysmal episode include

A
  • CHF, Pulmonary edema, MI, Vfib, Stroke
  • cardiac complications called Catecholamine cardiomyopathy (ventricular arrhythias)
74
Q

Lab results that would help confirm Pheochromocytoma Dx

A
  • increased urinary excretion of free catecholamines and their metabolites like:
  • Vanillylmandelic acid + Metanephrines
75
Q

Tx of pheochromocytomas

A
  • isolated benign tumors are surgically excised

(use of adrenergic blocking agents is vital for preventing HTN crisis during surgery)

  • multifocal lesions require long term medical Tx for HTN

Endocrine II - Exam 1 (6 decks)

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