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Endo II Exam 1 > Lecture 6: Adrenal Glands > Flashcards

Lecture 6: Adrenal Glands Flashcards

1
Q

Bilateral cortical hyperplasia of the adrenal glands is seen in which variants of Cushing Syndrome?

A

ACTH-dependent —> ACTH-producing pituitary adenoma or Ectopic ACTH-producing tumors

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2
Q

Atrophic adrenals would be expected in which variant of Cushing Syndrome?

A

“Iatrogenic” Cushing Syndrome from administration of exogenous glucocorticoids

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3
Q

What are the 2 most common underlying causes of ACTH-independent Cushing Syndrome?

A
  • Adrenal adenoma
  • Adrenal carcinoma
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4
Q

What are the serum levels of cortisol and ACTH like in adrenal adenomas/carcinomas?

A
  • ↑↑↑ serum cortisol
  • ↓↓↓ serum ACTH
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5
Q

What is the most common morphological alteration observed in the pituitary resulting from high levels of endogenous or exogenous glucocorticoids called?

A

Crooke hyaline change

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6
Q

Crooke hyaline change of the ACTH-producing cells of the anterior pituitary seen in Cushing Syndrome is the result of the accumulation of what?

A

Intermediate KERATIN filaments in the cytoplasm

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7
Q

What is a major morphological difference between adrenocortical adenomas and adrenocortical carcinomas?

A
  • Adenomas tend to be smaller w/ thin- or well-developed capsules
  • Carcinomas are MUCH larger and are UNencapsulated masses
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8
Q

Both benign and malignant adrenocortical adenomas are more common in which sex and age range?

A

Women in their 30s to 50s

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9
Q

Hypercortisolism causes selective atrophy of fast-twitch myofibers resulting in what clinical manifestations?

A
  • Decreased muscle mass
  • Proximal limb weakness
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10
Q

Which cause of Cushing Syndrome will have elevated levels of ACTH which is completely insensitive to low or high doses of exogenous dexamethasone?

A

Ectopic ACTH-producing tumors

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11
Q

Which 4 settings of HTN should raise suspicion of Primary Hyperaldosteronism (“Conn’s syndrome”)?

A
  • Refractory HTN
  • Adrenal mass + HTN
  • HTN at a young age
  • Severe HTN (>160/100 mmHg)
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12
Q

What is the most common underlying cause of primary hyperaldosteronism and what is seen morhphologically in the adrenal glands?

A
  • Bilateral idiopathic hyperaldosteornism (IHA)
  • Characterized by bilateral nodular hyperplasia of adrenal glands
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13
Q

Germline and somatic mutations of which gene are present in familial idiopathic hyperaldosteronism and some aldosterone-secreting adenomas?

A

KCNJ5 encoding a K+ channel

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14
Q

Glucocorticoid-remediable hyperaldosteronism is an uncommon cause of primary familial hyperaldosteronism and may be due to which genetic rearrangement and involving what chromosome?

A

Rearrangement on Cr. 8 placing CYP11B2 (gene encoding aldosterone synthase) under control of the ACTH-responsive CYP11B1 gene promoter

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15
Q

In glucocorticoid-remediable hyperaldosteronism what is the function of ACTH?

A

ACTH is able to stimulate the production of aldosterone synthase

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16
Q

Due to the unusual circumstance in glucocorticoid-remediable hyperaldosteronism where aldosterone production is under the control of ACTH, how can this production be suppressed?

A

Suppressible by dexamethasone

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17
Q

Secondary hyperaldosteronism occurs in response to conditions which do what?

A

Activate the RAAS

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18
Q

List 5 conditions where there is an increased activation of RAAS which may lead to secondary hyperaldosteronism?

A
  • Diuretic use
  • ↓ renal perfusion (i.e., arteriolar nephrosclerosis, renal a. stenosis)
  • Arterial hypovolemia (i.e., CHF, cirrhosis, nephrotic syndrome)
  • Pregnancy (estrogen-induced ↑ in plasma renin substrate)
  • Renin-secreting tumors
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19
Q

How does activity of the RAAS and levels of renin differ between primary and secondary hyperaldosteronism?

A
  • Primary assoc. w/ suppression of RAAS and ↓ renin
  • Secondary assoc. w/ activation of RAAS and ↑↑↑ renin
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20
Q

Do aldosterone-secreting adenomas typically produce visible enlargement?

A

NO, often small (<2 cm) and buried within the gland

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21
Q

What is a characteristic histological feature of aldosterone-secreting adenomas?

A

Eosinophilic, laminated cytoplasmic inclusions –> Spironolactone bodies

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22
Q

Is hypokalemia a mandatory feature of primary hyperaldosteronism?

A
  • NO, although many patients will be hypokalemic
  • Increasing numbers of patients who are normokalemic are being diagnosed
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23
Q

Diagnosis of primary hyperaldosteronism is confirmed by what screening test, and if postive, what test must be performed?

A
  • ↑↑↑ ratios of plasma aldosterone: plasma renin activity
  • Confirmed w/ aldosterone suppression test
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24
Q

What is the best therapy for primary hyperaldosteronism caused by an adenoma vs. bilateral hyperplasia?

A
  • Adenomas are amendable to resection
  • Bilateral hyperplasia is best managed w/ aldosterone antagonists i.e., spironolactone
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25
Q

Adrenocortical neoplasma associated with virilization are more likely to be of what type?

A

Androgen-secreting adrenal carcinomas

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26
Q

Androgen-secreting adrenal carcinomas often elaborate what other hormone?

A

Cortisol and are known as “mixed syndromes”

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27
Q

What is the inheritance pattern of the inherited metabolic errors causing congenital adrenal hyperplasia?

A

Autosomal recessive

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28
Q

Which enzyme involved in the biosynthesis of cortical steroids is most commonly seen as deficient?

A

21-hydroxylase deficiency

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29
Q

Which syndrome associated with 21-hydroxylase deficiency has a complete lack of the enzyme activity?

A

Salt wasting syndrome

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30
Q

What are the levels of mineralocorticoids, cortisol, and sex steroids like in salt wasting syndrome?

A
  • NO mineralocorticoids or cortisol
  • ↑↑↑ sex steroids
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31
Q

Salt wasting syndrome comes to attention soon after birth with what serum electrolyte abnormalities?

A
  • Salt wasting –> HYPOnatremia
  • HYPERkalemia
  • HYPOtension
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32
Q

How does the presentation of salt wasting syndrome differ in males vs. females at birth?

A
  • Females will present w/ easily recognizable virilization at birth
  • Males don’t usually come to clinical attention until 5-15 days after birth due to some salt-losing crisis
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33
Q

Patients w/ severe salt-wasting 21-hydroxylase deficiency will have adrenomedullary dysplasia which predisposes them to what long term consequences?

A
  • ↓ catecholamine secretion
  • HYPOtension
  • Circulatory collapse
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34
Q

Simple virilizing adrenogenital syndrome without salt wasting is associated with what type of 21-hydroxylase activity and how does this typically present clinically?

A
  • Partial lack of enzyme activity; some mineralcorticoids and small amount of cortisol, NOT enough to prevent ACTH overproduction
  • Presents as genital ambiguity
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35
Q

The most common form of 21-hydroxylase deficiency presents as nonclassic or late-onset adrenal virilism; what are the clinical manifestations?

A
  • May be virtually asymptomatic
  • Mild manifestations such a precocious puberty, acne and hirsutism at the time of puberty
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36
Q

In all cases of congenital adrenal hyperplasia what is the morphological change seen in the adrenals?

A

BILATERAL hyperplastic; some times ↑↑↑ 10-15x normal weights

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37
Q

Pt’s with CAH are treated with what?

A
  • Exogenous glucocorticoids, provides adequate levels AND suppresses ACTH levels
  • Mineralocorticoid supplementation given in salt-wasting variants
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38
Q

What are 2 means of diagnosis of congenital adrenal hyperplasia?

A
  • Serum 17-hydroxyprogesterone levels will be ↑↑↑
  • ACTH-stimulation test, which would normally ↑ glucocorticoids (but fails to do so in CAH)
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39
Q

Disseminated bacterial infection causing massive adrenal hemorrhagic necrosis and resultant primary acute adrenocortical insufficiency is known as what?

A

Waterhouse-Friderichsen Syndrome

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40
Q

Amyloidosis, sarcoidosis, and hemochromatosis may all lead to what type of adrenal dysfunction?

A

Primary adrenocortical insufficiency

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41
Q

In which 3 clinial settings does primary acute adrenocortical insufficiency most often occur?

A
  • As a crisis in pt with chronic adrenocortical insufficiency precipitated by any form of stress
  • Rapid withdrawl of steroids or failure to ↑ dose in response to acute stress
  • Massive adrenal hemorrhage, as occurring in newborns following prolonged/difficult delivery; pt’s on anticoagulant therapy who develop DIC; and in Waterhouse-Friderichsen Syndrome
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42
Q

Adrenal hemorrhage leading to primary acute adrenocortical insufficiency may occur in what 5 clinical settings?

A
  • Sepsis: Waterhouse Friderichsen syndrome
  • Neonatal period
  • Trauma
  • Postsurgical patients
  • Coagulopathy
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43
Q

Which 5 bacterial species may be associated with Waterhouse-Friderichsen Syndrome?

A
  • N. meningitidis septicemia
  • H. influenzae
  • Pseudomonas species
  • Pneumococci
  • Staphylococci
44
Q

What are the clinical signs/sx’s associated with Waterhouse-Friderichsen Syndrome?

A
  • Rapidly progressive HYPOtension —> Shock
  • DIC assoc. w/ widespread purpura, particularly of the skin
  • Massive bilateral adrenal hemorrhage –> rapid adrenocortical insufficiency
45
Q

Histological examination of the adrenals in Waterhouse-Friderichsen syndrome show hemorrhage that starts where and then travels how?

A

Starts within medulla near thin-walled venous sinusoids, then spreads peripherally into cortex, often leaving islands of recognizable cortical cells

46
Q

What are 6 signs/sx’s that should raise suspicion of acute adrenal insufficiency?

A
  • HYPOtension (refractory to volume repletion)
  • HYPOnatremia
  • HYPOglycemia
  • Abdominal pain
  • Fever
  • N/V
47
Q

90% of all cases of primary chronic adrenocortical insufficiency can be attributed to one of what 4 disorders?

A
  • Autoimmune adrenalitis
  • Tuberculosis
  • AIDS
  • Metastatic cancers
48
Q

Most common cause of primary chronic adrenocortical insufficiency in developed countries?

A

Autoimmune adrenalitis

49
Q

Most common cause of primary chronic adrenocortical insufficiency worldwide?

A

Tuberculosis —> Autoimmune

50
Q

Autoimmune polyendocrine syndrome type 1 is also know as what?

A

Autoimmune PolyEndocrinopathy, Candidiasis and Ectodermal Dystrophy (APECED)

51
Q

Autoimmune polyendocrinopathy syndrome type 1 (aka APECED) is characterized by what clinical findings?

A
  • Chronic mucocutaneous candidiasis
  • Abnormalities of skin, dental enamel, and nails (ectodermal dystrophy)
  • Organ-specific autoimmune disorders: autoimmune adrenalitis, hypoparathyroidism, idiopathic hypogonadism, and pernicious anemia
52
Q

Autoimmune polyendocrine syndrome type 1 is associated w/ mutations in which gene, on which chromosome?

A

AIRE on chromosome 21q22

53
Q

Autoantibodies against which 2 cytokines seen in autoimmune polyendocrinopathy syndrome type 1 are the reason for chronic mucocuntaneous infections?

A

IL-17 and IL-22

54
Q

When does autoimmune polyendocrine syndrome type 2 usually develop and what are the characteristic findings?

A
  • Starts in early adulthood
  • Combination of adrenal insufficiency (adrenalitis) + immune thyroiditis OR type 1 DM
55
Q

Which 3 infectious agents may cause primary chronic adrenocortical insufficiency?

A
  • Tuberculosis used to be most common cause
  • Histoplasma capsulatum
  • Coccidioides immitis
56
Q

AIDS patients are at risk for developing adrenal insufficiency from which 2 infections and as a complication of what?

A
  • CMV and Mycobacterium avium-intracellulare
  • Noninfectious (Kaposi Sarcoma)
57
Q

Metastatic neoplasms involving the adrenals and causing adrenocortical insufficiency most often arise from which 2 sites; what are some other sites which may be implicated?

A
  • Lungs and breast carcinomas = most common
  • May also be from GI carcinomas, malignant melanoma, and hematopoietic neoplasms
58
Q

What are 2 genetic causes of adrenal insufficiency?

A
  • Congenital adrenal hypoplasia
  • Adrenoleukodystrophy (X-linked recessive)
59
Q

What is the gross morphology and histology of the adrenal glands in primary autoimmune adrenalitis?

A
  • Irregularly SHRUNKEN glands
  • Scattered residual cortical cells in collapsed network of CT w/ a variable lymphoid infiltrate of the cortex
60
Q

Which type of inflammatory reaction will be seen in the adrenal glands affected by tuberculosis and fungal disease?

A

Granulomatous inflammation

61
Q

What are the initial manifestations (signs/sx’s) of primary chronic adrenocortical insufficiency (Addison’s disease)?

A

Progressive weakness and easy fatigability

62
Q

What are the signs/sx’s of primary chronic adrenocortical insufficiency as a result of corticosteroid and mineralocroticoid deficiency?

A
  • ↓ corticosteroids –> vague malaise, N/V, hypoglycemia, and refractory hypotension
  • ↓ mineralocorticoids –> hyperkalemia and hyponatremia
63
Q

Which type of adrenocortical insufficiency is associated with hyperpigmentation of the skin and which is not?

A
  • Primary adrenal disease will have hyperpigmentation
  • Adrenocortical insufficiency caused by primary pituitary or hypothalamic disease will not
64
Q

How does the presentation of adrenal carcinomas differ from that of adenoma?

A
  • Carcinomas are larger and produce mass effect causing compression/invasion of adjacent structures
  • Virilizing
65
Q

How does the production of cortisol, androgens, and aldosterone in secondary adrenocortical insufficiency differ from that of primary?

A
  • Will have deficient cortisol and androgens
  • But normal or near-normal synthesis of aldosterone
66
Q

How can an ACTH-stimulation test help you differentiate primary vs. secondary adrenocortical insufficiency?

A
  • Primary will have a reduced response to exogenous ACTH
  • Secondary will show a prompt rise in plasma cortisol levels
67
Q

Which 2 familial cancer syndromes are associated with a predisposition for developing adrenocortical carcinomas?

A
  • Li-Fraumeni syndrome, due to germline TP53 mutations
  • Beckwith-Wiedemann syndrome a disorder of epigenetic imprinting
68
Q

Adrenocortical carcinomas have a strong tendency to invade which structures?

A
  • Adrenal vein
  • Vena cava
  • Lymphatics —> regional and periaortic nodes = common
69
Q

Distant hematogenous spread by adrenocortical carcinomas to where is common; what is the prognosis of these neoplasms?

A
  • Lungs and other viscera
  • Median pt survival = about 2 years
70
Q

Which is more common, primary adrenocortical carcinomas or metastases to the adrenal cortex?

A

Metastases

71
Q

Cortical and medullary neoplasms may undergo necrosis and cystic degeneration and may present as what type of lesion?

A

“Nonfunctional cysts”

72
Q

Adrenal myelolipomas are unusual benign lesions composed of what?

A

Mature fat + hematopoietic cells (i.e., bone marrow)

73
Q

What is the 10% rule associated with Pheochromocytomas (5 of them)?

A
  • 10% are extra-adrenal (paraganglioma)
  • 10% are bilateral
  • 10% in kids
  • 10% are malignant
  • 10% are NOT associated with HTN
74
Q

What % of pheochromocytomas are now recognized as harboring germline mutations and as such are familial?

A

25%

75
Q

What are the 3 groups of extra-adrenal paraganglia based on anatomic distribution?

A
  1. Branchiomeric
  2. Intravagal
  3. Aorticosympathetic
76
Q

What are 4 familial syndromes which are associated with pheochromocytomas and extra-adrenal paragangliomas?

A
  • MEN-2A
  • MEN-2B
  • NF-1
  • Von Hippel-Lindau (VHL)
77
Q

How does the patient presentation and location of pheochromocytomas in pt’s harboring germline mutations differ from that of sporadic types?

A
  • Pt’s are typically younger
  • More often harbor bilateral disease
78
Q

The histological pattern in pheochromocytomas is composed of clusters of polygonal to spindle-shaped chromaffin or chief cells surrounded by what?

A

Supporting sustentacular cells, creating small nests or alveoli (zellballen) that are supplied by rich vascular network

79
Q

The cytoplasm of cells within a pheochromocytoma has a finely granular appearance and is best demonstrated with what stain due to granules containing catecholamines?

A

Silver Stain

80
Q

What stain can be used for visualization of the peripheral sustentacular cells of a pheochromocytoma?

A

Stain w/ antibodies against S-100

81
Q

Which histological feature of pheochromocytomas reliably predicts clinical behavior?

A

NONE

82
Q

What is the definitive diagnosis of malignancy in pheochromocytomas based on?

A

Exclusively on the presence of metastases

83
Q

What is the dominant clinical manifestation of pheochromocytomas and what is the classic triad?

A
  • HTN which can be either chronic or paroxysmal
  • Triad = Headache + Palpitations + Diaphoresis
84
Q

What are some acute complications of pheochromocytomas which are associated with catecholamine surges?

A
  • CHF
  • Pulmonary edema
  • MI
  • Ventricular fibrillation
  • CVA
85
Q

What is a chronic cardiac complication which may arise in the setting of a pheochromocytoma?

A

Catecholamine cardiomyopathy

86
Q

What is the laboratory diagosis of pheochromocytomas based on?

A

↑ urinary excretion and plasma free catecholamines + their metabolites i.e., vanillylmandelic acid and metanephrines

87
Q

What 3 features of adrenal incidentalomas impact the appropriate managment of the mass?

A
  • SIZE: >4cm = more likely to be carcinoma
  • Positive functional assays: i.e., dexamethasone suppression test for hypercortisolism or urinary metanephrines for pheochromo.
  • CT enhancement characteristics
88
Q

How do endocrine tumors arising in the context of MEN syndromes differ from their sporadic counterparts in terms of age, organs involved, behavior, and foci?

A
  • Tend to occur at younger age
  • Arise in multiple endocrine organs, either synchronously or metachronously
  • Typically preceded by asymptomatic stage of hyperplasia
  • Even in one organ, tumors are often multifocal
  • Are usually more aggressive and recur
89
Q

What are the “3 P’s” of MEN, type 1?

A
  • Primary hyperparathyroidism
  • Pancreatic endocrine tumors
  • Pituitary adenomas
90
Q

What is the initial manifestation of MEN 1 in most patients, appearing in almost all patients by age 40-50?

A

Primary hyperparathyroidism

91
Q

Which tumors of MEN-1 are the leading cause of morbidity and mortality due to their aggressiveness and metastases?

A

Pancreatic endocrine tumors

92
Q

What is the most frequent pituitary adenoma encountered in MEN-1?

A

Prolactinoma (lactotroph); may also see somatotroph adenoma (GH)

93
Q

What is the most common site of gastrinomas present in pt’s with MEN-1?

A

Duodenum; may also have synchronous duodenal + pancreatic tumors

94
Q

MEN-1 is due to germline mutations in the MEN1 tumor suppressor gene which encodes what protein?

A

Menin

95
Q

What is the triad of MEN-2A?

A
  • Pheochromocytoma
  • Medullary thyroid carcinoma
  • Parathyroid hyperplasia

*There are more ‘A’s’ in Type 2 ‘A’

96
Q

Which tumor is seen in almost 100% of patients with MEN-2A?

A

Medullary carcinoma of the thyroid

97
Q

The medullary thyroid carcinoma encountered in MEN-2A are usually multifocal and are virtually always associated with foci of what?

A

C-cell hyperplasia in the adjacent thyroid

98
Q

MEN-2A is caused by which mutation and on what chromosome?

A

Germline gain-of-function mutations in RET on Cr. 10q11.2

99
Q

What is the triad of MEN-2B?

A
  • Pheochromocytomas
  • Medullary thyroid carcinomas
  • Mucosal neuromas (distinguishing feature from MEN-2A)
100
Q

Which MEN syndrome is associated with a marfanoid habitus, characterized by long axial skeletal features and hyperextensible joints?

A

MEN-2B

101
Q

How is the germline mutation associated with MEN-2B distinct from that of MEN-2A?

A

MEN-2B caused by germline mutation leading to a single amino acid change in RET (specific point mutation)

102
Q

Familial medullary thyroid cancers are a variant of MEN-2A; how are they different?

A
  • Develop at an older age
  • Do not have the other clinical manifestation assoc. w/ MEN-2A
  • Typically follow a more indolent course
103
Q

About 1/3 of sporadic medullary thyroid carcinomas harbor the identical point mutation of RET encountered in MEN-2B and follow what type of course?

A

More aggressive disease and adverse prognosis

104
Q

Diagnosis via screening of at-risk family members for which MEN syndrome is important?

A

MEN-2A due to medullary thyroid carcinoma being life-threatening, and can be prevented by prophylactic thyroidectomy

105
Q

The pineocytes of the pineal gland are epithelial cells with what 2 functions?

A

Photosensory and neuroendocrine functions

106
Q

Majority of tumors arising in the pineal gland are of what type?

A
  • Germ cell tumors i.e., germinomas, embryonal carcinomas; choriocarcinomas and mixtures of the 2

Endo II Exam 1 (6 decks)

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