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Endocrine System > The Pathology of Adrenal Gland > Flashcards

The Pathology of Adrenal Gland Flashcards

1
Q

What is adrenal hyperfunction knwon as?

A

Hyperadrenalism

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2
Q

What are the three distinctive hyperadrenal clinical syndromes?

A
  1. Cushing syndrome
  2. Hyperadosetronism
  3. Adrenogenital or Virilzing syndromes
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3
Q

What characterizes Cushing syndrome?

A

Characterized by an excess in cortisol

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4
Q

What characterizes hyperaldosteronism?

A

Characterized by chronic excess aldosterone secretion

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5
Q

What causes adrenogenital or virilizing syndromes?

A

Excess of androgens

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6
Q

What causes hypercortisolism and Cushing syndrome?

A

Any condition that produces an elevation in glucocorticoid levels (exogenous or endogenous)

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7
Q

What could be the cause of exogenous hypercortisolism?

A

Result of administration of exogenous glucocorticoids (iatrogenic)

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8
Q

What does exogenous hypercortisolism lead to?

A

Leads to bilateral adrenal atrophy, steroid suppress ACTH secretion (negative feedback)

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9
Q

What are the causes of endogenous hypercortisolism? (3)

A
  1. Primary hypothalamic-pituitary diseases associated with hypersecretion of ACTH
  2. The secretion of ectopic ACTH by non-pituitary neoplasms
  3. Primary adrenocortical neoplasms (adenoma or carcinoma) and rarely primary cortical hyperplasia
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10
Q

What are primary hypothalamic-pituitary diseases associated with hypersecretion of ACTH like? (3)

A
  1. Accounts for 70% of cases of Cushing syndrome
  2. 4x higher in women
  3. Age group: 20s to 30s
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11
Q

What causes primary hypothalamic-pituitary diseases associated with hypersecretion of ACTH?

A

Corticotroph microadenoma or corticotroph cell hyperplasia

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12
Q

What are non-pituitary neoplasms that are associated with ACTH ectopic secretion like?

A
  1. Account for 10% of causes of Cushing syndrome
  2. Elevated ACTH –> Bilateral cortical hyperplasia
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13
Q

What causes non-pituitary neoplasms that are associated with ACTH ectopic secretion?

A

Lung cancer (small cell carcinoma)
Medullary carcinomas of the thyroid

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14
Q

What are the primary adenocortical neoplasms and primary cortical hyperplasia like? (2)

A
  1. Account for 15 to 20% of cases of endogenous Cushing syndrome
  2. Adrenals function autonomously (ACTH-independent Cushing syndrome, or adrenal Cushing syndrome)
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15
Q

What causes the primary adenocortical neoplasms and primary cortical hyperplasia?

A

1.A unilateral adrenocortical neoplasm: benign or malignant
2. Primary cortical hyperplasia: 2 variants –> macronodules of varying sizes or micronodules

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16
Q

What is considered a MACROnodule in primary cortical hyperplasia?

A

3mm or greater in diameter

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17
Q

What does the morphology of Cushing syndrome depend on?

A

It depends on the cause of hypercortisolism

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18
Q

What is the morphology of Cushing syndrome due to cortical atrophy? (2)

A
  1. suppression of endogenous ACTH
  2. Lack of stimulation of the zona fasciculata and zona reticularis by ACTH
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19
Q

What is the morphology of Cushing syndrome due to diffuse hyperplasia? (3)

A
  1. Patients with ACTH-dependent Cushing syndrome
  2. Both glands are enlarged (up to 30g)
  3. The yellow colour of diffusely hyperplasticity glands derives from the presence of lipid-rich cells, which appear vacuolated under the microscope
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20
Q

What is the morphology of Cushing syndrome due to macronodular or micronodular hyperplasia?

A

Cortex is replaced by darkly pigmented macronodules (> 3mm) or micronodules (1 to 3mm)

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21
Q

What is the morphology of Cushing syndrome due to Adenoma or carcinoma?

A
  1. Adrenocortical adenomas are yellow tumors surrounded by thin or well-developed capsules (weigh less than 30g)
  2. On microscopic examination, they are composed of cells similar to those encountered in the normal zona facsiculata
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22
Q

What are the cells upon microscopic examination in adenomas or carcinomas of Cushing syndrome?

A

Neoplastic cells vacuolated because of the presence of intracytoplasmic lipids

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23
Q

What is the progression of signs and symptoms of Cushing syndrome?

A

They represent an exaggeration of the known actions of glucocorticoids (they develop gradually)

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24
Q

What are the early manifestations of Cushing syndrome? (5)

A
  1. Hypertension
  2. Weight gain
  3. Muscle weakness
  4. Secondary diabetes
  5. Osteoporosis
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25
Q

What is the characteristic of weight gain in Cushing syndrome?

A

Characteristic centripetal distribution of adipose tissue becomes apparent, with resultant truncal obesity, fascial “moon face,” and accumulation of fat in the posterior neck and back “buffalo hump”

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26
Q

What is primary hyperaldosteronism? (2)

A
  1. Autonomous overproduction of aldosterone
  2. Suppression of the RAAS and decreased plasma renin activity
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27
Q

What are the potential causes of primary hyperaldosteronism? (3)

A
  1. Bilateral idiopathic hyperaldosteronism (60%)
  2. Adrenocortical neoplasm (35%)
  3. Familial hyperaldosteronism (rare)
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28
Q

What is bilateral idiopathic hyperaldosteronism?

A

Bilateral nodular hyperplasia of the adrenal glands
Germline mutation in the KCNJ5 gene

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29
Q

What is the function of the KCNJ5 gene?

A

Encodes a potassium channel protein that is expressed in the adrenal gland

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30
Q

What is adrenocortical neoplasm?

A
  1. Solitary aldosterone-secreting adenoma (Conn syndrome)
  2. Somatic mutations of KCNJ5
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31
Q

What is familial hyperaldosteronism?

A

The genetic defect that leads to overactivity of the aldosterone synthase gene, CYP11B2

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32
Q

What is secondary hyperaldosteronism?

A

Aldosterone release occurs in response to activation of the renin-angiotensin system (characterized by increased levels of plasma renin)

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33
Q

What is secondary hyperaldosteronism associated with?

A
  1. Decreased renal perfusion –> arteriolar nephrosclerosis, renal artery stenosis
  2. Arterial hypovolemia and edema –> CHF, cirrhosis, nephrotic syndrome
  3. Pregnancy –> caused by estrogen-induced increases in plasma renin substrate
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34
Q

What is the clinical hallmark of hyperaldosteronism?

A

Hypertension

35
Q

Why is hypertension the hallmark of hyperaldosteronism?

A

Aldosterone increases sodium absorption and secretion of potassium and hydrogen ions (distal tubes and collecting duct)
Increased absorption of sodium expands plasma volume, leading to hypertension

36
Q

What are the long-term effects of the hyperaldosteronism induced hypertension?

A
  1. Cardiovascular compromise
  2. Hypokalemia
  3. Variety of neuromuscular manifestations, including weakness, paresthesias, visual disturbances, and frank tetany
37
Q

What is the cardiovascular compromise of hyperaldosteronism-induced hypertension?

A

LEft ventricular hypertrophy and reduced diastolic volumes
Increase in the prevalence of adverse events such as stroke and MI

38
Q

What causes the hypokalemia of the hyperaldosteronism-induced hypertension?

A

Renal potassium wasting

39
Q

What are adrenogenital syndromes?

A

Excess sex steroids with hyperplasia of nothing adrenal glands

40
Q

What are the causes of congenital adrenal hyperplasia?

A

Inherited 21-hydroxylase deficiency (common cause 90%)
Impaired synthesis of cortisol and aldosterone

41
Q

How does impaired synthesis of cortisol lead to CAH?

A

Deficiency of cortisol leads to increased (compensatory) ACTH secretion (due to the absence of feedback inhibition), which results in bilateral adrenal hyperplasia

42
Q

How does impaired synthesis of aldosterone lead to CAH?

A

Deficiency of synthesis of aldosterone leads to salt wasting with hyponatremia, hyperkalemia, hypovolemia, and shock (neonatal adrenal crisis)

43
Q

What are the clinical features of congenital adrenal hyperplasia (CAH)?

A

Signs of masculinization in females
Enlargement of the external genitalia and precocious puberty

44
Q

What are the signs of masculinization seen in females (CAH)?

A

Clitoral hypertrophy and pseudohermaphroditism in infants to oligomenorrhea, hirsutism, and acne in postpubertal girls

45
Q

What are the patterns of adrenal insufficiency?

A
  1. Primary hypoadrenalism
  2. Secondary hypoadrenalism
46
Q

What are the subtypes of primary hypoadrenalism?

A

Acute (adrenal crisis)
Chronic (Addison disease)

47
Q

What is secondary hypoadrenalism?

A

Decreased stimulation of the adrenals resulting from ACTH deficiency

48
Q

What are the causes acute adrenocortical insufficiency?

A
  1. Waterhouse-Friderichse syndrome
  2. Stress in the pituitary with underlying chronic adrenal insufficiency
  3. Sudeen withdrawal from long-term corticosteroid therapy
49
Q

What are the causes of Waterhouse-Friderichse syndrome?

A
  1. Neisseria meningitidis septicemia (common)
  2. Pneumococci, Haemophilus influenza (less common)
  3. Endotoxin-induced vascular injury with associated disseminated intravascular coagulation
50
Q

What is Chronic adrenocortical insufficiency?

A

Addison disease, progressive destruction of the adrenal cortex

51
Q

What is Addison’s disease caused by?

A
  1. Autoimmune adrenalitis
  2. Infections
  3. Metastatic cancers
52
Q

What is autoimmune adrenalitis that could leadd to Addison disease?

A

Occurs in DEVELOPED countries
Autoimmune polyendocrine syndromes: APS1 caused by mutations in the AIRE gene or ASP2

53
Q

Where is the AIRE gene found?

A

On chromosome 21

54
Q

What are the infections that could cause Addison’s disease?

A

Occur in the DEVELOPING countries
Tuberculosis, AIDS (opportunistic pathogens)
Fungi

55
Q

What fungal infection could lead to Addison’s disease?

A

Histoplasma capsulatum

56
Q

What types of .metastic cancers could lead to Addison’s disease?

A

Carcinomas of the lung and breast

57
Q

What is secondary adrenocortical insufficiency?

A

Any disorder of the hypothalamus and pituitary that reduces the output of ACTH (metastatic cancers, infections, infractions or irradiation)

58
Q

Can ACTH develop alone ?

A

Most of the time, it develops alone, but in some instances, it is one of the panhypopituitarisms associated with multiple pituitary hormone deficiencies

59
Q

What is tertiary adrenal insufficiency?

A

Any disorder that interferes with the secretion of ACTH-releasing hormone (CRH) by the hypothalamus can lead to inadequate ACTH secretion and, thus, adrenal insufficiency

60
Q

What are the clinical features of adrenal insufficiency? (5)

A
  1. Patients typically present with fatigue, weakness, and GIT disturbances
  2. Hypotension, hyponatremia, hypovolemia, hyperkalemia –> decreased aldosterone activity
  3. Hypoglycemia due to glucocorticoid deficiency and impaired gluconeogenesis
  4. Weakness
  5. Hyperpigmentation -> due to increased ACTH –> increased melanocytic production
61
Q

WHat is the adrenal medulla?

A
  1. Composed of NCC-derived chromaffin cells
  2. Main physiologic source of catecjolamines
62
Q

WHat is pheochromocytoma?

A

Neoplasm composed of chromaffin cells (synthesize and release catecholamines)

63
Q

What are the clinical features of the pheochromocytoma due to?

A

Increased serum catecholamines

64
Q

What are the clinical features of pheochromocytoma?

A

Episodic hypertension, headache, palpitations, tachycardia, and sweating

65
Q

What is the rule of 10s when it comes to pheochromocytoma?

A

10% of pheochromocytomas are extra-adrenal, organ of Zuckerkandl, and the carotid body (paragangliomas)

10% of adrenal pheochromocytomas are bilateral

10% of adrenal pheochromocytomas are malignant

10% of adrenal pheochromocytomas are familial (recently modified to 25%)

66
Q

What are the mutations which lead to familial pheochromocytomas?

A

Germline mutations of the RET (type 2 MEN syndromes), NF1, VHL

67
Q

What are the gross features of pheochromocytomas?

A

Small, circumscribed lesions confined to the adrenal
Large hemorrhagic masses weighing several kilograms

68
Q

What is the cut-surface of small pheochromocytomas?

A

Yellow-tan, well-defined lesion that compress the adjacent adrenal

69
Q

What is the cut surface of larger lesions?

A

Hemorrhagic, necrotic, and cystic typically affect the adrenal gland

Incubation of the fresh tissue with potassium dichromate solutions turns the tumor dark brown

70
Q

Wha are the microscopic features of pheochromocytomas cells?

A

Polygonal to spindle-shaped chromaffin cells, compartmentalized into small nests (Zellballen) by a rich vascular network

71
Q

What are the microscopic features of the cytoplasm of pheochromocytoma?

A

Finely granular appearance

72
Q

What are the microscopic features of the nuclei of pheochromocytomas?

A

Often pleomorphic

73
Q

What are the clinical features of pheochromocytomas?

A

Hypertension
Associated with tachycardia, palpitations, headache, sweating, tremor
Cardiac complications

74
Q

What are the cardiac complications associated with pheochromocytomas?

A
  1. Catecholamine cardiomyopathy
  2. Catecholamine-induced myocardial instability
  3. Ventricular arrhythmias
75
Q

What is the lab diagnosis of pheochromocytomas based on?

A

Demonstration of increased urinary excretion of free catecholamines and their metabolites

76
Q

What are examples of the catecholamine metabolites?

A

Vanillylmandelic acid anemia metanephrines

77
Q

What is neuroblastoma?

A

The second most common solid malignancy of childhood after brain tumors, accounting for 7 to 10% of all pediatric neoplasms

78
Q

Where do neuroblastomas aruse from?

A

Neural crest-derived cells in the sumpathetic ganglia and adrenal medulla

79
Q

What do neuroblastomas secrete?

A

Secrete catecholamines, whose metabolites can be used for screening patients

80
Q

What is the pathogenesis of neuroblastomas?

A

Germline mutations in the ALK gene –> familial predisposition to neuroblastoma

Somatic gain-of-function ALK mutations are also observed in 8 to 10% of sporadic neuroblastomas

81
Q

What are important diagnostic features of neuroblastomas?

A

Elevated blood levels of catecholamines
Elevated urine levels of catecholamine metabolites (VMA and HVA)

82
Q
A
83
Q
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Endocrine System (25 decks)

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