Adrenal Gland Pathology Flashcards

1
Q

What does the cortical zone look like?

A
  • Yellow color
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2
Q

What are the components of the cortical zone (from out to in)?

A
  • Zona glomerulosa
  • Zona fasciculata
  • Zona reticularis
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3
Q

What does the zona glomerulosa secrete?

A
  • Aldosterone
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4
Q

What does the zona fasciculata secrete?

A
  • Cortisol
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5
Q

What does the zona reticularis secrete?

A
  • Weak androgens
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6
Q

What does the medulla secrete?

A
  • Catecholamines
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7
Q

What does the medulla look like?

A
  • Dark brown
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8
Q

What do the adrenals look like in ACTH dependent cushing syndrome?

A
  • Bilateral cortical hyperplasia
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9
Q

What are the symptoms of primary hyperaldosteronism (Conn’s syndrome)?

A
  • Hypertension
  • Hypokalemia
  • Hypomagnesemia
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10
Q

What is seen in the hypertension with primary hyperaldosteronism?

A
  • Refractory hypertension
  • Adrenal mass and hypertension
  • Hypertension at a young age
  • Severe hypertension
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11
Q

What are some things that disrupt the RAAS causing secondary hyperaldosteronism?

A
  • Diuretic use
  • Decreased renal perfusion
  • Arterial hypovolemia
  • Pregnancy
  • Renin-secreting tumors
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12
Q

What can be used to treat hypertension in an aldosterone-secreting adenoma?

A
  • Spironolactone
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13
Q

What is congenital adrenal hyperplasia?

A
  • Inherited error of metabolism (AR)
  • Defective enzyme responsible for steroidogenesis
  • Impaired feedback to hypothalamus/pituitary, with resultant hyperplasia
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14
Q

What enzyme is deficient in a majority of cases of congenital adrenal hyperplasia?

A
  • 21-hydroxylase
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15
Q

What is salt wasting syndrome?

A
  • Complete lack of 21-hydroxylase enzyme
  • No mineralocorticoids
  • No cortisol
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16
Q

What is the presentation that is shared in both males and females for salt wasting syndrome?

A
  • Hyponatremia
  • Hypokalemia
  • Hypotension
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17
Q

What is a presentation that is seen in only females with salt wasting syndrome?

A
  • Virilization (seen at birth)
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18
Q

What is a long term consequence of adrenomedullary dysplasia?

A
  • Hypotension
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19
Q

What is simple virilizing syndrome?

A
  • Partial lack of 21-hydroxylase enzyme
  • Some mineralocorticoids
  • Small amounts of cortisol, not enough to prevent ACTH overproduction
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20
Q

What is seen in simple virilizing syndrome?

A
  • Virilization
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21
Q

What is nonclassic/late onset adrenal virilism?

A
  • Partial lack of 21-hydroxylase (more than simple virilizing syndrome)
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22
Q

What is seen in nonclassic/late onset adrenal virilism?

A
  • Precocious puberty

- Acne and hirsutism at time of puberty

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23
Q

How is the diagnosis of CAH made?

A
  • Look at serum 17-hydroxyprogesterone
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24
Q

What is the treatment for CAH?

A
  • Give glucocorticoids
  • It replenishes cortisol
  • Provides negative feedback for ACTH suppression
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25
Q

What are some causes of primary adrenocortical insufficiency?

A
  • Loss of cortical cells

- Defect in hormonogenesis

26
Q

What are some causes of secondary adrenocortical insufficiency?

A
  • Hypothalamic-pituitary disease

- HPA suppression by extra-adrenal steroid source

27
Q

What are some causes of primary acute adrenocortical insufficiency?

A
  • Adrenal crisis
  • Rapid withdrawal of steroids
  • Massive adrenal hemorrhage (Waterhouse-Friderichsen syndrome)
  • Stress, infection, trauma, burns
28
Q

What happens to the adrenal glands on corticosteroids?

A
  • Atrophy
29
Q

What are the clinical symptoms of acute adrenal insuffiency?

A
  • Hypotension
  • Abdominal pain
  • Fever
  • N/V
  • Hyponatremia
  • Hypoglycemia
30
Q

What are some symptoms of primary chronic adrenocortical insuffiency?

A
  • Long duration of malaise, fatigue
  • Anorexia and weight loss
  • Joint pain
  • Hyperpigmentation of skin
31
Q

What was the most common cause of Addison’s disease when it was initially found?

A
  • TB
32
Q

What is the most common cause of Addison’s disease in developed countries and worldwide?

A
  • Autoimmune
33
Q

What causes autoimmune polyendocrine syndrome type 1?

A
  • Mutation in AIRE gene
34
Q

What is seen in autoimmune polyendocrine syndrome type 1?

A
  • Adrenalitis
  • Parathyroiditis
  • Hypogonadism
  • Pernicious anemia
35
Q

What is seen in autoimmune polyendocrine syndrome type 2?

A
  • Adrenalitis
  • Thyroiditis
  • Type 1 diabetes mellitus
36
Q

What is apeced?

A
  • Has same symptoms of autoimmune polyendocrine syndrome type 1
  • Also has misshapen teeth and nail beds
37
Q

What are individuals with apeced more likely to have?

A
  • Mucocutaneous candidiasis

- Ectodermal dystrophy

38
Q

What are some ways to check for adrenocortical insufficiency?

A
  • Random cortisol

- ACTH-stimulation test

39
Q

What is the size that signifies it is a carcinoma?

A
  • > 200 grams
40
Q

What are some features that are more commonly seen with carcinoma?

A
  • Invasion of adjacent structures

- Virilization

41
Q

What is the adrenal medulla derived from?

A
  • Comprised of chromaffin cells deriving from the neural crest
42
Q

What is the adrenal medulla responsible for?

A
  • Catecholamine secretion

- Epi and Norepi

43
Q

What is a pheochromocytoma?

A
  • Patients can present with profound hypertension

- Results from catecholamine secretion from the tumor

44
Q

What is the 10% rule with pheochromocytoma?

A
  • 10% are extra-adrenal
  • 10% are bilateral
  • 10% are in kids
  • 10% are malignant
  • 10% are not associated with hypertension
45
Q

What is the 25% rule with pheochromocytoma?

A
  • 25% are familial
46
Q

What is the presentation of a pheochromocytoma?

A
  • Hypertension is present in >90% of cases
  • Headache
  • Palpitations
  • Diaphoresis
47
Q

What are some complications with pheochromocytomas?

A
  • Acute: related to catecholamine surges

- Chronic: cardiomyopathy

48
Q

How is a pheochromocytoma diagnosed?

A
  • Urine and plasma metanephrines
49
Q

What is a myelolipoma?

A
  • Benign (fat or bone marrow)
  • Vary in size
  • Can present with hemorrhage
50
Q

What is an adrenal incidentaloma?

A
  • Incidentally discovered adrenal nodule
51
Q

What is done for an adrenal incidentaloma?

A
  • CT enhancement characteristics
  • Positive functional assays ( Dexa suppression test or pheochromocytoma)
  • Look at size
52
Q

What comprises MEN type 1?

A
  • Primary hyperparathyroidism
  • Pancreatic endocrine tumors
  • Pituitary adenomas (lactotrophs and somatotrophs)
53
Q

What is the germline mutation in MEN type 1?

A
  • MEN tumor suppressor gene (menin)
54
Q

What comprises MEN type 2A?

A
  • Pheochromocytoma
  • Medullary thyroid carcinoma
  • Parathyroid hyperplasia
55
Q

What germline gain of function is seen in MEN type 2A?

A
  • RET proto-oncogene
56
Q

What comprises MEN type 2B?

A
  • Medullary thyroid carcinoma
  • Pheochromocytoma
  • Mucosal neuromas
57
Q

What germline gain of function is seen in MEN type 2B?

A
  • RET proto-oncogene
58
Q

What are some generalities of MEN syndromes?

A
  • Patients develop tumors at younger ages
  • Tumors are more likely to be bilateral/multiple
  • Preceding hyperplasia is often seen
  • Tumors tend to be aggressive and reccurent
59
Q

What are some tumors of the pineal gland?

A
  • Germ cell tumors
  • Pineocytoma
  • Pineoblastoma
60
Q

What is the pineal gland responsible for?

A
  • Melatonin secretion