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Endocrine Two > Adrenal Gland Pathology > Flashcards

Adrenal Gland Pathology Flashcards

1
Q

Specific steroid and location of GC

A

Cortisol, adrenal cortex–>zona fasciculata

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2
Q

Specific steroid and location of MC

A

Aldosterone, adrenal coretx –> zona glomerulosa

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3
Q

Specific steroid and location of sex steroids

A

Estrogen, androgens - zona reticularis

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4
Q

Specific steroid and location of chromaffin cells

A

epinepherine, cats - adrenal medulla

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5
Q

Two types of endogenous, ACTH-dependent Cushing Syndrome

- Which is most common and W:M

A
  • Cushing Disease - pituitary adenoma **70% of cases; 4:1 W:M
  • Ectopic corticotropin syndrome (ACTH)
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6
Q

Four types of endogenous, ACTH-Independent Cushing Syndrome

A
  1. Adrenal adenoma or carcinoma
  2. Macronodular hyperplasia
  3. Primary pigmented nodular adrenal disease
  4. McCune-Albright Syndrome
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7
Q

Majority of ACTH-secreting pituitary adenomas are micro- or macroadenomas?

A

Microadenomas.

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8
Q

You see cortical cell hyperplasia, think what?

A

Think discrete adenoma.

  • Primary OR
  • Secondary (hypothalamic CRH producing tumor)
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9
Q

If you see a small cell carcinoma of the lung, be on the lookout for?

A

ACTH-dependent Cushing Syndrome - the nonpituitary tumor secreting ectopic ACTH

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10
Q

If there is a neuroendocrine neoplasm and resultant increased ACTH and hypercortisolism, what is it probably ectopically secreting?

A

CRH

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11
Q

Most common types of nonpituitary ectopic-ACTH secreting tumors (ACTH-dependent Cushing)

A
  1. SMALL CELL CARCINOMA
  2. carinoids, medularry thyroid carcinoma, islet cell tumors
  3. CRH-secreting neuroendocrine
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12
Q

Most common types of primary adrenal neoplasms (ACTH-independent Cushing Syndrome)

A

Adrenal adenoma (10%), carcinoma (5%)

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13
Q

Hallmark of ____?

↑ cortisol and ↓ ACTH

A

Primary Adrenal neoplasm (adenoma or carcinoma)

ACTH‐independent Cushing syndrome

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14
Q

If there is marked hypercortisolism, think what?

A

cortical carcinomas (not adenoma or hyperplasias)

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15
Q

Most hyperplastic adrenals are ACTH dependent or independent?

A

ACTH depenent

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16
Q

Early v. late characteristic features of Cushing Syndrome

A

Early - HTN, wt gain

Late - moon facies, buffalo hump, truncal obesity (central pattern of fat deposition)

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17
Q

List four major catabolic effects of Cushing Syndrome

A

– Skin is thin, fragile, and easily bruised
– Poor wound healing
– Cutaneous striae are particularly common in the abdominal area
– Osteoporosis

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18
Q

What affect does hypercortisolism (Cushing Syndrome) have on type 2 fast twitch myofibers?

A

ATROPHY - decreased muscle mass and proximal limb weakness

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19
Q

Presentation of SECONDARY DIABETES in Cushing Syndrome and why?

A

Presents with Hyperglycemia, glucosuria and polydipsia

– Glucorticoids induce gluconeogenesis and inhibit the uptake of glucose by cells

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20
Q

Cushing Syndrome and the immune system

A

IMMUNE SUPPRESSION

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21
Q

Explain the (1) laboratory diagnosis of Cushing Syndrome and (2) its cause

A

(1)
• Increased 24‐hour urine free‐cortisol*
• Loss of normal diurnal pattern of cortisol secretion
(2)
• Determining the cause of Cushing syndrome:
– Serum ACTH
– Dexamethasone suppression test: Urinary excretion of 17‐hydroxycorticosteroids after administration of dexamethasone

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22
Q

A pt presents with HTN, think…

A

Primary hyperaldosteronism. Autonomous over production of aldosterone. Suppression of RAS

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23
Q
  • Pts older with (less severe) hypertension

* Bilateral nodular hyperplasia of the adrenal glands

A

Bilateral Idiopathic Hyperaldosteronism

• Most common cause of primary hyperaldosteronism

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24
Q

Most likely type of adrenocortical neoplasm that causes primary hyperaldosteronism. Sex and age

A

Adenoma (conn Syndrome)

W>M (2:1), middle age

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25
Q
  • Familial hyperaldosteronism
  • Ch 8 rearrangement that places the gene for aldosterone synthase under the control of the ACTH responsive gene promoter
A

Glucocorticoid-Remediable Hyperaldosteronism

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26
Q

Labs for:
– ACTH stimulates the production of aldosterone
– Suppressible by dexamethasone

A

Glucocorticoid-Remediable Hyperaldosteronism

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27
Q

Aldosterone released in response to activation of the renin‐angiotensin system by ↑ plasma renin

A

Secondary hyperaldosteronism

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28
Q

In what three instances is secondary hyperaldosteronism typically seen?

A
  1. Decreased renal perfusion
  2. Arterial hypovolemia and edema
  3. Pregnancy
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29
Q

Long term effects of HTN on CV system (hyperaldosteronism)

A

LVH, decreased diastolic volumes, stroke, MI

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30
Q

A person presents with weakness, paresthesias, visual disturbances, and occasionally tetany. What notable electrolyte is abnormal and what is the cause?

A

Hypokalemia due to hyperaldosteronism.

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31
Q

Screening and confirmation lab test for hyperaldosteronism

A
  • Screening test: Elevated ratio of plasma aldosterone concentration to plasma renin activity
  • Confirmation test: Aldosterone suppression test
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32
Q

Treatment of hyperaldosteronism due to:

  • adenoma
  • bilateral hyperplasia
  • Secondary hyperaldosteronism
A
  • adenoma - resection
  • bilateral hyperplasia - pharm (aldosterone antagonist like spironolactone)
  • Secondary hyperaldosteronism - tx underlying cause
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33
Q

Deficiency or total lack of an enzyme involved in the biosynthesis of cortical steroids, esp. cortisol. Presents with androgen excess, with or without aldosterone and glucocorticoid deficiency.

A

Congenital Adrenal Hyperplasia

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34
Q

Increased androgens in CAH = ?

Impaired aldosterone secretion = ?

A

virilization

Salt wasting

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35
Q

Three distinct syndromes in 21-hydroxylase deficiency

A

– Salt‐wasting syndrome
– Simple virilizing adrenogenital syndrome without salt wasting
– Nonclassic or late‐onset adrenal virilism

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36
Q

Genetics of 21-hydroxylase deficiency

A

CYP21A2 mutation

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37
Q

Inability to convert progesterone into deoxycorticosterone. What is this called and what are the clinical effects?

A

Salt-wasting syndrome - salt wasting (hyponatremia and hyperkalemia) → acidosis, hypotension, cardiovascular collapse, and possibly death

38
Q

When is Salt Wasting Syndrome recognized?

A

– Virilization recognized in the female at birth or in utero

– Males come to clinical attention 5‐15 days later because of some salt‐losing crisis

39
Q

What presents with:
• Presents as genital ambiguity
• ~1/3 of 21‐hydroxylase deficiency
• Progressive virilization

A

Simple Virilizing Andrenogenital Syndrome Without Salt Wasting

40
Q

• Most common form of CAH
• Only a partial deficiency in 21‐hydroxylase
function
• Asymptomatic
• Hirsutism, acne, and menstrual irregularities

A

Nonclassic or Late-onset Adrenal Virilism

41
Q

Neonate is born with ambiguous genetalia. What should be suspected immediately?

A

CAH

42
Q

What enzyme deficiency does this female have?
• Clitoral hypertrophy and pseudohermaphroditism in infants
• Oligomenorrhea, hirsutism, and acne in postpubertal

A

21‐hydroxylase deficiency

43
Q

What enzyme deficiency does this male have?
• Enlargement of the external genitalia and other evidence of precocious puberty in prepubertal patients
• Oligospermia in older males

A

21‐hydroxylase deficiency

44
Q

Why does severe 21‐hydroxylase deficiency have such a strong effect on the adrenal medulla? What results?

A

ADRENOMEDULLARY DYSPLASIA
GC required to make cats (Epi/NE).
Low cortisol levels → adrenomedullary dysplasia (↓ catecholamine secretion) → hypotension and circulatory collapse

45
Q

Treatment for CAH (two)

A
  1. exogenous GC

2. MC supplementation in salt-wasting variants of CAH

46
Q

What occurs in this setting? Exogenous corticosteroids, in whom rapid withdrawal of steroids or failure to increase steroid doses in response to an acute stress

A

Primary Acute Adrenocortical Insufficiency

47
Q

Massive adrenal hemorrhage damages the adrenal cortex and causes Primary Acute Adrenocortical Insufficiency - what settings can this damage occur in?

A

–Newborns after traumatic delivery and hypoxia
– Anticoagulant therapy
– Postsurgical patients who develop DIC
– Disseminated bacterial infection (Waterhouse‐Friderichsen syndrome)

48
Q

What is this?
• Rapidly progressive hypotension leading to shock
• DIC associated
with widespread purpura
• Rapidly developing adrenocortical insufficiency associated with massive bilateral adrenal hemorrhage
• Fatal, unless promptly recognition and treated

A

Waterhouse-Friderichsen Syndrome

49
Q

Bug associated with Waterhouse-Friderichsen Syndrome.

A

Neisseria meningitidis septicemia

50
Q

What is this - progressive destruction of adrenal cortex. If caused by autoimmune adrenal insufficiency, then seen more common in whites and women. Sx when 90% of cortex is lost.

A

Primary Chronic Adrenocortical Insufficiency (Addison Disease)

51
Q
  • Autoimmune destruction of steroidogenic cells

* Autoantibodies to 21‐hydroxylase and 17‐ hydroxylase have been detected

A

Autoimmune Adrenalitis, 60-70% of Addison Disease

Occur in either APS1 and APS2

52
Q

• Candidiasis
• Abnormalities of skin, dental enamel, and nails (ectodermal
dystrophy)
• Autoimmune dz/polyendocrine disorders (i.e. Autoimmune Adrenalitis)

A

APS1

53
Q

Genetics of APS1

A

Mutation in AIRE gene on ch21

– autoimmunity

54
Q

Autoantibodies against what two IL’s to eliminate defense against fungal infections?

A

IL‐17 and IL‐22

55
Q
  • Usually starts in early adulthood

* Presents as a combination of adrenal insufficiency and autoimmune thyroiditis or type 1 diabetes

A

APS2

56
Q
  • Early manifestations: progressive weakness and easy fatigability
  • GI disturbances – Anorexia, N/V/D, weight loss
  • Primary adrenal disease: get hyperpigmentation of the skin
  • Hypoglycemia can occur – impaired gluconeogenesis
A

Addison Disease

57
Q

Where on the body is hyperpigmentation form Addison Diseaemost prominent?

A

Sun‐exposed areas and pressure points (e.g. neck, elbows, knees, and knuckles)

58
Q

Why does this hyperpigmentation occur in Addison Disease?

A

– Results from elevated levels of pro‐opiomelanocortin (POMC)
• Originates in ant-pit and is precursor of ACTH and melanocyte stimulating hormone (MSH)

59
Q

A person with Addison has previously undergone some sort of stress ( infection, trauma, surgical procedure). Presents with intractable vomiting, abdominal pain, hypotension, coma, and vascular collapse. What do they have and what therapy must begin immediately to avoid death?

A

acute adrenal crisis. begin Corticosteroid therapy

60
Q

Electrolyte/labs in Addison, due to decreased MC activity

A

hyperkalemia, hyponatremia

volume depletion, hypotension

61
Q

Define secondary adrenocortical insufficiency

A

Any disorder of the hypothalamus and pituitary (met cancer, infection, infarction, radiation) that REDUCES the output of ACTH.

62
Q

Main differences between Addison Disease and secondary adrenocortical insufficiency.

A

No hyperpigmentation, no hyperklaemia/hyponatremia.

63
Q

Lab dx for secondary adrenocortical insufficiency

A

**Labs - low cortisol and androgen output, but normal or near normal aldosterone.

(give exogenous ACTH –> prompt rise in cortisol)

64
Q

Major bacteria possibly implicated in Waterhouse

A

Neisseria meningitidis septicemia

– Also see with Pseudomonasspecies, pneumococci, Haemophilus influenzae, or even staphylococci

65
Q

Adrenocortical neoplasms: type and age

A

Adenomas = carcinomas in adults

Carcinomas > adenomas in kids

66
Q

Familal or sporadic - adrenocortical carcinomas

A

both.

Familial = Li‐Fraumeni syndrome and Beckwith‐Wiedemann syndrome

67
Q

Mutation in Li-Fraumeni Syndrome

A

Germline TP53 mutation

68
Q

More likely adenoma or carcinoma:
• Hyperaldosteronism and Cushing syndrome
• Virilizing neoplasm

A
  1. More likely from a functional adenoma

2. More likely carcinoma

69
Q

Benign lesions composed of mature fat and marrow in adrenal gland.

A

Adrenal myelolipomas

70
Q

– Frequent abdominal imaging led to discovery many incidental adrenal masses
– Asymptomatic
– ~4% of the population
– Majority are small, nonsecreting cortical adenomas

A

“adrenal incidentaloma”

71
Q

Rare cause of surgically correctable hypertension

– ~2/3 have “paroxysmal” episodes associated with sudden rise in blood pressure and palpitations (can be fatal)

A

Pheochromocytoma

72
Q

What type of neoplasm is a pheochromocytoma?

A

composed of chromaffin cells in the adrenal medulla

73
Q

“Rule of 10s” for pheochromocytomas

A 
– 10% are extra‐adrenal
– 10% bilateral
– 10% malignant
– 10% don’t have hypertension
(10% calcify, kids, bilateral)
74
Q

What can precipitate paroxysmal episodes in pts with pheochromocytoma?

A 
– Emotional stress
– Exercise
– Changes in posture
– Palpation in the region of the tumor
– Patients with urinary bladder paragangliomas = paroxysm during micturition
75
Q

Bilateral, younger onset, occurs in 1/2 of people with pheos and paragangliomas - are they familial or sporadic?

A

familial

76
Q

RET gene - syndrome and lesion

A

MEN-2A/2B; pheochromocytoma

77
Q

SDHD/SDHB gene - syndrome and lesion

A

Familial paraganglioma 1 and 4; pheochromocytoma and paraganglioma

78
Q

Dx of a pheo

A

Increased urinary excretion of free catecholamines and metabolites:
• Vanillylmandelic acid and metanephrines

79
Q

Tx of a pheo

A

Surgical excision, tx HTN

80
Q

Parathyroid, Pancreas, Pituitary (PRL-oma), and possible:
• Duodenum is the most common site of gastrinomas
• Carcinoid tumors
• Thyroid adenomas
• Adrenocortical adenomas
• Lipomas

A

MEN-1 “wermer Syndrome”

81
Q

MEN1 genetics

A

Mutation in MEN1 tumor suppressor gene - encodes menin.

82
Q

What do these three characterize?
• Pheochromocytoma (40‐50%)
• Medullary carcinoma of the thyroid (~100%)
• Parathyroid hyperplasia (10‐20%)

A

Sipple Syndrome, MEN-2A

83
Q

MEN-2A genetics

A

germline mutation in RET proto-oncogene; ch10

84
Q

• Medullary carcinoma of the thyroid (~100%), most aggressive
• Pheochromocytomas
• Neuromas or ganglioneuromas of the skin, oral mucosa,
eyes, respiratory tract, and gastrointestinal tract
• Marfanoid habitus

A

MEN-2B

85
Q

Genetics of MEN-2B - offer at risk family members prophylactic what?

A

germline mutation in single aa in RET - prophylactic thyroidectomy

86
Q
  • Variant of MEN‐2A
  • Only medullary thyroid cancer
  • Pts older
  • More indolent course
A

Familial medullary thyroid cancer

87
Q

– Photosensory and neuroendocrine functions
– Secretes melatonin
Tumors are rare

A

Pineal Gland

88
Q

If tumor present (rare), what type in the pineal gland?

A

– Most commonly germ cell tumors - Germinomas, embryonal carcinomas; choriocarcinomas, teratomas
– Pinealomas - Pineoblastomas and pineocytomas

89
Q

do adrenal adenomas suppress ACTH?

A

no

90
Q

Four syndromes in which paragangliomas form.

A

VHL, Familial paraglanglioma 1,3,4

Endocrine Two (16 decks)

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