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D&T3 Exam 1 > Adrenal-graffeo > Flashcards

Adrenal-graffeo Flashcards

1
Q

MEN I (Wermer’s syndrome)

Location

A

Pituitary

Parathyroid (primary hyperparathyroidism MC)

Pancreatic Islet cell tumors

Duodenal gastrinoma

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2
Q

Congenital Adrenal Hyperplasia

Defect

A

Defect in enzyme in cortisol synthesis

Decreased cortisol causes increased ACTH

Results: adrenocortical hyperplasia

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3
Q

Cushings

S&Sx

Later

A

Truncal obesity, “moon” facies, “buffalo hump”

Atrophy of FAST TWITCH muscles (II)– able to fire quickly for short bursts:: slow twitch for continuous, extended muscle contractions over a long time ====

Get dec muscle mass and proximal limb weakness

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4
Q

Pheochromocytoma

S&Sx

A

Tachycardia

Palpitations

Tremor

Apprehension

PAIN in abdomen or chest

N/V

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5
Q

Hypercortisolism

Cushings

Endogenous cause

Primary adrenocortical neoplasm or hyperplasia

MC

A

Most: neoplasms (adenomas or carcinomas)

Adults: adenomas and carcinomas

Kids: mostly carcinomas

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6
Q

Chronic insufficiency

S&Sx

A

Onset: insidious

Progressive weakness and easy fatigability

GI: anorexia, N/V, weight loss, diarrhea

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7
Q

Pheochromocytoma

Rx

A

Single: surgery

Bilateral/multiple: medical

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8
Q

Pheochromocytoma

Labs

A

Inc urinary excretion of

Free catecholamines

Their metabolites VMA

Dopamine

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9
Q

Cushings

S&Sx

Early

A

Hypertension and weight gain

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10
Q

Chronic insufficiency

Due too

A

Autoimmune (60%)

Autoimmune polyendocrine syndrome type 1 (APS1)

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11
Q

Cushings

Clinical findings

A

Hyperglycemia, glucosuria, poydipsia

Cutaneous STRIAE and osteoporosis

Hirsutism, mental disturbances, POOR WOND HEALING

FACIAL PLETHORA, DEC LIBIDO, THIN SKIN, MENSTRUAL IRREGULARITY, EASY BRUISING, WEAKNESS

POSTERIOR NECK ADIPOSITY

OSTEOPENIA AND FRACTURE

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12
Q

Adrenogenital syndromes

Congenital Adrenal Hyperplasia

21-Hydroxylase Deficiency

Can cause

A

Inc androgen activity

In 1/3, na wasting (complete lack)–get hyponatremia, hyperkalemia, hypotension, or even death

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13
Q

MEN IIa (Sipple’s Syndrome)

Genetic mutation

A

RET protooncogene on chromosome 10

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14
Q

Chronic insufficiency

Morphology

Primary autoimmune

A

Irregular shrunken adrenals

Variable lymphoid infiltrate

Medulla usually preserved

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15
Q

Pheochromocytoma

S&Sx

BP

A

Hypertension: 2/3 chronic sustained, may be labile

1/3–paroxysmal may occur: abrupt, precipitous elevation

Inc risk of AMI, Hebert failure, renal injury and CVA’s

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16
Q

Adrenal

Zona fasciculata secretes

A

Cortisol and androgens

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17
Q

Cushings

Ectopic-ACTH

A

ACTH elevated

Neither low or high does dexamethasone supresses

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18
Q

Neuroblastoma

Morphology

A

Small round blue cell

HOMER WRIGHT PSUDOROSETTES

May have large cells resembling neurons if better differentiated (ganglioneuromas)

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19
Q

Adrenogenital syndromes

Congenital adrenal hyperplasia

Rx

A

Exogenous glucocorticoids: suppress ACTH, risk of acute adrenal insufficiency

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20
Q

Pheochromocytoma

Benign

A

May have capsular and vascular invasion

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21
Q

Acute adrenocortical insufficiency

Waterhouse Friederichsen Syndrome

A

Massive adrenal hemorrhage

Classically N meningitidis

Could be pseudomonas, pneumococci, H. Influenzae

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22
Q

Chronic insufficiency

Acute adrenal crisis

Can cause death if…

A

Corticosteroids and mineralocorticoids not replaced immediately

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23
Q

Adrenal insufficiency

Autoimmune polyendocrine syndrome type 1 (APS1)

A

Chronic mucocutaneous candidiasis

Ectodermal dystrophy (abnormalities of skin, dental enamel and nails)

Autoimmune adrenalitis, autoimmune hypo parathyroid is, idiopathic hypogonadism, pernicious anemia

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24
Q

Chronic insufficiency

S&Sx

Primary (adrenal)

A

Hyperpigmentation due to precursors of ACTH (POMC)

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25
Q

Acute adrenocortical insufficiency

Exogenous steroids

A

If rapidly withdrawn or figure to inc in stress

Remember adrenals ATROPHIED so no normal response to ACTH

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26
Q

Hypercorisolism=

A

Cushings

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27
Q

MEN IIa (Sipple’s Syndrome)

Parathyroid Hyperplasia (10 to 20%)

A

Hypercalcemia

Renal stones

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28
Q

Neuroblastoma

S&Sx

Older

A

Metastases: hepatomegaly, ascites, and bone pain

90% produce catecholamines (HTN

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29
Q

Endocrine Neoplasia syndromes

MEN

A

Tumors at younger age than sporadic

Arise in multiple endocrine organs synchronously or metachronously

Multi focal in same organ

More aggressive

Preceded by hyperplasias

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30
Q

Acute Adrenocortical Insufficiency

Can develop from…

A

Chronic insufficiency

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31
Q

Chronic insufficiency

APS2

A

Similar to APS1 but lacks candidiasis, skin changes and auto immune hypoparathyroidism

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32
Q

Hyperaldosteronism

A

Leads to na retention, k excretion with resultant hypertension and hypokalemia

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33
Q

Neuroblastoma

Labs

A

Inc serum catecholamines

Inc urinary VMA and HVA (more specific)

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34
Q

Cushings

Morphology

Main lesions

A

Pituitary and adrenal gland

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35
Q

Chronic insufficiency

Morphology

Mets

A

Adrenals enlarged by neoplasm

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36
Q

Pheochromocytoma

S&Sx

Sudden death

A

May be due to inc catecholamines

Cause myocardial instablity

Result: ventricular arrhythmias

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37
Q

Adrenal insufficiency

Decreased ACTH

Primary pituitary

A

Sheehan’s Syndrome

Nonfunctional Pituitary Adenoma

Lesions of Hypothalamus or Suprasellar

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38
Q

Hyperaldosteronism

Primary due to hyperplasia

A

Diffuse or irregular

Proliferation of Zona Glomerulosa

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39
Q

Addison’s

A

Chronic adrenocortical insufficiency

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40
Q

Adrenal neoplasms

Adenoma and carcinoma

A

Both usually UNILATERAL

Similar (encapsulated, yellow tumors) but carcinoma larger

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41
Q

Hypercortisolism

Cushings

Endogenous cause

Primary adrenocortical neoplasm or hyperplasia

Results in…

A

Inc glucocorticoids===dec acth

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42
Q

Hypercortisolism

Cushings

Endogenous cause

Secretion of ectopic ACTH by neuroendocrine tumors

And dexamethasone

A

Inc ACTH NOT suppressed by high dose dexamethasone

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43
Q

Pheochromocytoma

Morphology

A

10% multicentric and/or bilateral

Hemorrhage, necrotic, and cystic

Stain with silver stain

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44
Q

Congenital Adrenal Hyperplasia

A

Group of disorders

Autosomal RECESSIVE

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45
Q

Neuroblastoma

Prognosis

A

Stage and age most important

Genetics: deletion of short arm of chromosome 1 (aggressive)

Amplification of N-NYC oncogene (POOR prognosis)

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46
Q

Hyperaldoseronism

Can cause…

A

Inc aldosterone leading to na retention and k excretion

Hypertension and hypokalemia

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47
Q

Adrenal insufficiency

Pathogenesis

Primary (adrenal)

A

Dec glucocorticoids

Inc ACTH (End-Organ Failure)

48
Q

Hyperaldosteronism

S&Sx

A

Primary: adenoma

Hypertension and hypokalemia

Female

Middle age adult

49
Q

Secondary Hyperaldosteronism

Results in

A

Inc aldosterone

50
Q

Adrenal

Zona reticularis

A

Estrogens and androgens

51
Q

Cushings is an xs of…

A

Cortisol

52
Q

Hypercortisolism

Cushings

Endogenous cause

Secretion of ectopic ACTH by neuroendocrine tumors

Results in

A

Inc ACTH causes nodular adrenocortical hyperplasia==resulte in inc GLUCOCORTICOIDS

53
Q

Hyperaldosteronism

Glucocorticoid-suppressible

Genetics

A

Primary familial HA

Chimeric gen CYP11B1 (11-B-hydrosylase) and CYP11B2 (aldosterone synthase)

54
Q

Hyperaldosteronism

Glucocorticoid-Suppressible

Histology

A

Derangement in zonation

Hybrid cells (zona Glomerulosa and zona fasiculata)

HYBRID steroids

55
Q

Chronic insufficiency

Morphology

Secondary (pituitary)

A

Atrophy

56
Q

Adrenal neoplasms

Carcinoma

A

Similar to adenoma but larger, usually has capsule

57
Q

Adrenogenital syndromes

Congenital Adrenal Hyperplasia

MC

A

21-hydroxylase Deficiency

Carrier frequency 1:120

Hispanics and Ashkenazi Jews

58
Q

Adrenal

Zona glomerulosa secretes

A

Cortisol and aldosterone

59
Q

Pituitary changes

Crooke’s hyaline

Histology

A

Intermediate keratin filaments

Normal granular basophilic cytoplasm of ACTH producing cells replaced by homogenous lightly basophilic material

60
Q

MEN IIa (Sipple’s Syndrome)

Pheochromocytoma (40-50%)

A

Benign or malignant

61
Q

Adrenocortical neoplasms

Morphology

Adenoma

A

Most NONFUNCTIONAL

Generally small (1-2cm)

62
Q

Hyperaldosteronism

Rare cause

A

Glucocorticoid-suppressible

63
Q

Acute adrenocortical insufficiency

Massive adrenal hemorrhage

Due to

A

Newborns due to BIRTH TRAUMA (coagulopathy-prothrombin deficiency)

Anticoagulant therapy

DIC Pregnancy

Waterhouse Friederichsen Syndrome

64
Q

Hypercortisolism

Cushings

Endogenous cause

Primary adrenocortical neoplasm or hyperplasia

Hyperplasia

A

Less common: may be AD inherited trait

65
Q

Pheochromocytoma

Aka

A

10%tumor

10% bilateral

10% familial

10% malignant

10% not ass with HTN

66
Q

Chronic insufficiency

Other causes

A

Infections: TB, fungi (H. Capsulatum, Coccidiodes immitis)

Metastatic disease (lung, breast, etc.)

67
Q

MEN IIb (or MEN III)

Can see

A

Thyroid medullary CA and Pehochromocytoma as in MEN IIa

Neuromas and/or ganglioneuromas

NO parathyroid disorder

68
Q

MEN IIa

Aka

A

Sipple’s syndrome

69
Q

Pheochromocytoma

Malignancy

A

Based exclusively on METS

70
Q

Secondary Hyperaldosteronism

Inc renin due to

A

CHF

Dec renal perfusion (arteriolar nephrosclerosis, renal artery stenosis)

Hypoalbuminemia

Pregnancy (estrogen causes release of plasma renin substrate)

71
Q

Cushings

Pituitary cause

A

Not suppressed by low dose dexamethasone

Suppressed by HIGH dose dexamethasone

ACTH Elevated

72
Q

MEN I (Wermer’s syndrome)

Pancreatic islet cell tumor (2nd most common)

A

Insulin, glucagon, gastrin, somatostatin, VIP

Multiple

73
Q

Adrenal medulla

Inner action

A

Preganglionic nerves from sympathetic nervous system stimulate secretion

74
Q

Neuroblastoma

S&Sx

<2 yrs old

A

Protuberant abdomen

Abdominal mass

Fever and weight loss

75
Q

Adrenal medulla

A

Synthesizes and secretes catecholamines from chromaffin cells

76
Q

Pituitary changes

Crooke’s hyaline

Cause

A

Results from high levels of cortisol

77
Q

Adrenal

Medulla

A

Catecholamines

78
Q

Chronic adrenocortical insufficiency=

A

Addisons

79
Q

Chronic insufficiency

Autoimmune polyendocrine syndrome type 1 (APS1)

Mutation in

A

APS1 caused by mutations in the autoimmune regulator gene (AIRE) chromosome 21q22

Transcriptions factor, failure of self-tolerance resulting in auto immunity

80
Q

Neuroblastoma

Origin and stats

A

15% of all CHILDHOOD CANCER deaths

Origin: neural crest:: may arise anywhere in sympathetic chain

81
Q

MEN IIa (Sipple’s Syndrome)

Common presentation

A

Think medulla

Thyroid medullary CA

Pheochromocytoma (adrenal medulla)

82
Q

Adrenocortical neoplasms

Morphology

Carcinoma

A

Rare

Any age

Likely FUNCTIONAL

Large, invasive, met by lymphatic &/or blood

Median survival–2 years

83
Q

Hypercortisolism

Cushings

Causes of xs cortisol

A

Exogenous: IATROGENIC (MC!!!! From giving exogenous glucocorticoids

Endogenous: primary hypothalamic-pituitary ass hypersecretion of ACTH, Primary adrenocortical neoplasm or hyperplasia, secretion of ectopic ACTH by neuroendocrine tumors

84
Q

Chronic insufficiency

S&Sx

Dec mineralocorticoids cause

A

Hyperkalemia, hyponatremia, volume depletion, hypotension

Hypoglycemia

Small heart

85
Q

MEN I

Aka

A

Wermer’s Syndrome

86
Q

Cushings

Morphology

Exogenous cause

Adrenal

A

Inc STEROIDS causes dec ACTH==results in atrophy of the. Adrenals

87
Q

Chronic insufficiency

Morphology

TB and Fungus

A

Granulomatous reaction

88
Q

Conn syndrome

A

Presence of solitary aldosterone secreting adenoma

Primary hyperaldosteronism

89
Q

Hyperaldosteronism

Glucocorticoid-Suppressible

Dexamethasone

A

Under control of ACTH

Glucocorticoids WILL suppress (dexamethasone)

90
Q

Hyperaldosteronism

Morphology

Primary due to adenoma (80%)

A

Usually solitary

Does not suppress ACTH–like seen with cushings; so adjacent gland shows NO atrophy

91
Q

Adrenal medulla

Derived from

A

Neural crest

92
Q

MEN IIb (or MEN III)

A

GENETICALLY distinct from MEN IIa

Different RET protooncogene mutation

93
Q

Chronic insufficiency

Acute adrenal crisis

A

Intractable vomiting

Abdominal pain

Hypotension

Coma

Vascular collapse

94
Q

Adrenogenital syndromes

Types

A

Adrenocortical neoplasms (MC virilizing carcinomas)

Congenital adrenal hyperplasias

95
Q

Pheochromocytoma

Symptoms brought on by…

A

Emotional stress

Exercise

Changes in posture

Palpation in the region of the tumor

96
Q

MEN I (Wermer’s syndrome)

Gene mutation

A

Mutations in tumor suppressor gene MEN1 (protein is menin)

97
Q

MEN I (Wermer’s syndrome)

Pituitary

A

Any type of adenoma

98
Q

Adrenal insufficiency

Pathogenesis

Primary (pituitary)

A

Dec ACTH causes dec glucocorticoids

99
Q

Secondary Hyperaldosteronism

Cause

A

Inc renin

Extra-adrenal

100
Q

Cushings

Morphology

Endogenous

A

Results in cortical hyperplasia

EITHER PITUITARY ACTH OR ECTOPIC STIMULATES THE ADRENAL AND RESULTS IN BILATERAL NODULAR ADRENOCORTICAL HYPERPLASIA

Enlargement of both glands, thickened yellow cortex, nodule formation

101
Q

Hyperaldosteronism

Rx

Adenomas

A

Surgical correction of HYPERTENSION possible

102
Q

Adrenal insufficiency

Primary (adrenal)

A

Adrenal

Chronic- addison’s

Acute

103
Q

MEN IIa (Sipple’s Syndrome)

Thyroid medullary CA (100%)

A

Ass with foci of C-CELL HYPERPLASIA

AGGRESSIVE

104
Q

Adrenal myelolipoma

A

Rare benign adrenal lesion

Composed of fat and hematopoietic cells

105
Q

Adrenocortical neoplasms

Classification

A

Based on CLINICAL not morphology

Functional (secrete hormone) or non functional

106
Q

Hypercortisolism

Cushings

Endogenous cause

Primary hypothalamic-pituitary ass with hypersecretion of ACTH

A

Cushing disease

MC endogenous causes

3rd to 4th decade

Inc acth causes bilateral nodular cortical hyperplasia=results in hypercortisolism

Most small adenoma

107
Q

Adrenal neoplasms

Adenoma

A

Encapsulated, yellow tumors

108
Q

3 layers of adrenal

A

Zona glomerulosa

Zona fasciculata

Zona reticularis

Medulla

109
Q

Acute adrenocortical insufficiency

Major complication

A

Massive adrenal hemorrhage

110
Q

Primary Hyperaldosteronism

Cause

A

Adrenal

Neoplasm (usually adenoma) or hyperplasia

Inc aldosterone

Dec renin

Solitary aldosterone secreting adenoma=conn syndrome

111
Q

Adrenogenital syndromes

Congenital adrenal hyperplasia

Morphology

A

Inc ACTH causes bilateral nodular adrenal hyperplasia

Pituitary:: hyperplasia of corticotrophs

112
Q

Hyperaldosteronism

Rx
Hyperplasias

A

Treated medically

113
Q

MEN I (Wermer’s syndrome)

MC presentation

A

Hyperparathyroidism

Adenoma or hyperplasia

114
Q

Cushings

Adrenal cause

A

ACTH DECREASED

Neither low or high dose dexamethasone supresses

115
Q

Hypercortisolism

Cushings

Endogenous cause

Secretion of ectopic ACTH by neuroendocrine tumors

Cause

A

Least common

Due to SMALL CELL CA OF LUNG

D&T3 Exam 1 (19 decks)

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