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Endocrine & Reproduction II Exam #2 > Adrenal Pathology and MEN Syndromes > Flashcards

Adrenal Pathology and MEN Syndromes Flashcards

1
Q

ACTH dependent Cushing syndrome =

ACTH independent Cushing syndrome =

A

ACTH dependent Cushing syndrome = secondary hyperadrenalism

ACTH independent Cushing syndrome = usually a primary hyperadrenal state

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2
Q

When would you see BL cortical hyperplasia of the adrenal glands?

A

ACTH dependent Cushing syndrome

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3
Q

What are 3 features of Primary aldosteronism (“Conn’s syndrome”)?

A

HTN

  • refractory HTN
  • adrenal mass and HTN
  • HTN at a young age
  • severe HTN (>160/100 mmHg)

Hypokalemia

Hypomagnesemia

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4
Q

What causes secondary aldosteronism?

A

Overactivation of the RAAS system

  • diuretic use
  • decreased renal perfusion
  • arterial hypovolemia
  • pregnancy
  • renin-secreting tumors
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5
Q

Aldosterone-secreting adenoma are of what size?

Who do they affect?

What is a classic finding on histology?

There is a high incidence of what with these adenomas?

A

Often very small (< 2 cm)

Young patients: 30s-40s

Spironolactone bodies

High incidence of ischemic heart disease

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6
Q

What is a pituitary cause of Adrenogenital syndromes (“adrenal virilization”)?

What are 2 adrenal causes?

A

Pituitary: ACTH stimulation of androgens (Cushing disease)

Adrenal:

  • Primary adrenal neoplasm (carcinoma > adenoma)
  • Congenital adrenal hyperplasia (CAH)
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7
Q

What is the inheritance of CAH?

What is the underlying metabolic cause?

How does the hyperplasia occur?

A

AR

There is a deficiency of 21-hydroxylase, an enzyme responsible for steroidogenesis (inherited error of metabolism)

There is impaired feedback to the hypothalamus/pituitary, which causes the hyperplasia of the adrenals.

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8
Q

What causes Salt wasting syndrome?

What are some symptoms/presentations in men and women? (3)

What about in females only? (1)

What is the long-term consequence of adrenomedullary dysplasia?

A

21-hydroxylase deficieny leads to no mineralcorticoids and no cortisol.

Males and females:
Salt wasting -> hyponatremia
Hyperkalemia
Hypotension

Females:
Virilization (seen at birth)

Hypotension is the long-term consequence.

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9
Q

What causes Simple virilization syndrome (w/o salt wasting)?

What is a major symptom?

A

Partial 21-hydroxylase deficiency - some mineralocorticoids and small amount of cortisol, but not enough to prevent ACTH overproduction.

Virilization

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10
Q

What causes Non-classic/Late onset adrenal virilism?

What are 2 symptoms?

A

Partial 21-hydroxylase deficiency

Precocious puberty
Acne and hirsutism at the time of puberty

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11
Q

What marker is used to diagnose CAH?

What is done at birth to test for it?

A

Serum 17-hydroxyprogesterone

Heel stick of the baby

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12
Q

What is done for therapy for CAH? (2)

A

Glucocorticoids - it replenishes cortisol and provides a negative feedback for ACTH suppression (no further overstimulation of androgen production).

May give mineralocorticoids as needed.

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13
Q

What are potential causes of primary adrenal insufficiency? (2)

Secondary adrenal insufficiency? (2)

A

Primary

  • loss of cortical cells
  • defect in hormonogenesis

Secondary

  • hypothalamic-pituitary disease
  • HPA suppression by extra-adrenal steroid source
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14
Q

Primary acute adrenocortical insufficiency is an…

What causes it?

What is a concerning complication?

A

Adrenal crisis!

Rapid withdrawal of steroids

Massive adrenal hemorrhage (Waterhouse -Friderichsen syndrome)

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15
Q

Relative adrenal insufficiency causes a…

What are 3 changes seen?

A

Dramatic adrenal crisis!

Hypotension, hyponatremia and hyperkalemia

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16
Q

What adrenal cause might lead to sepsis?

A

Waterhouse Friderichsen syndrome - N. meningitidis infection

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17
Q

What symptoms would be seen in acute adrenal insufficiency? (6)

A 
Hypotension (refractory to volume repletion)
Abdominal pain
Fever
N/V
Hyponatremia
Hypoglycemia
18
Q

What are symptoms of primary chronic adrenocortical insufficiency? (4)

A

Long duration of malaise, fatigue

Anorexia and weight loss

Joint pain

Hyperpigmentation of skin

19
Q

How does hyperpigmentation occur as a result of elevated CRH?

A

CRH stimulates the production of POMC (pre-pro-hormone synthesized in the anterior pituitary) which is then cleaved to make ACTH and MSH.

20
Q

Which disease is the same as Addison disease?

A

Primary chronic adrenocortical insufficiency

21
Q

What was the most common cause of Addison’s disease at the time of its initial description?

What is the most common cause in developed countries now?

A

Tb

Autoimmune mediated

22
Q

> 70% of all cases of primary hypoadrenalism in the western world =

A

Autoimmune adrenalitis - Autoimmune polyendocrine syndrome types 1 and 2

23
Q

Autoimmune polyendocrine syndrome type 1 is caused by what?

What are the classic symptoms/findings? (6)

A

Mutation in AIRE gene

APECED

  • adrenalitis
  • parathyroiditis
  • hypogonadism
  • pernicious anemia
  • mucocutaneous candidiasis (Ab’s against IL-17 and IL-22)
  • ectodermal dystrophy
24
Q

What are the 3 classic symptoms/findings in Autoimmune polyendocrine syndrome type 2?

A

Adrenalitis
Thyroiditis
T1DM

25
Q

Which 4 symptoms does a lack of corticosteroids in adrenocortical insufficiency cause?

Which 2 symptoms occur due to the lack of mineralocorticoids?

A 
Corticosteroids:
Vague malaise
N/V
Hypoglycemia
Refractory hypotension

Mineralocorticoids:
Hyperkalemia
Hyponatremia

26
Q

How can you test for adrenocortical insufficiency? (2)

A

Random cortisol

ACTH-stimulation test

27
Q

Which are bigger: adenoma or carcinoma?

A

Carcinoma > adenoma

Typically > 200 gm

28
Q

What is the presentation of adrenal adenomas and carcinomas?

Are they functional?

What are 2 features unique to carcinomas?

A

Both tend to be incidental findings.

Yes, both are functional.

Compression/invasion of adjacent structures and virilization.

29
Q

What is the job of the adrenal medulla?

A

To secrete catecholamines under sympathetic control.

30
Q

Pheochromocytoma commonly presents with which symptom?

What is the 10% rule? (5)

How many are familial?

A

Profound HTN

10% rule:

  • 10% are extra-adrenal (paraganglioma)
  • 10% are BL
  • 10% in kids
  • 10% are malignant (can only tell by mets)
  • 10% are not associated w/ HTN

25% are familial.

31
Q

Pheochromocytomas can be chronic or…

What is the classic triad?

What are acute and chronic complications?

How are they diagnosed?

A

Chronic or paroxysmal

HA, palpitations and diaphoresis

Acute - related to catecholamine surges
Chronic - CM

Urine and plasma metabolites

32
Q

What is a myelolipoma made of? Benign or malignant?

How big are they?

What can they present with?

A

Benign - fat and bone marrow.

They come in various sizes.

May present w/ hemorrhage.

33
Q

What is an adrenal incidentaloma?

Proper management of them depends on what factors? (3)

A

Incidental growths seen on imaging intended for something else.

> 4 cm.
Positive functional assays (dexamethasone suppression test for hypercortisolism or Pheochromocytoma)
CT enhancement characteristics

34
Q

4 MEN type 1 syndromes

A

Primary hyperparathyroidism

Pancreatic endocrine tumors

Pituitary adenomas (lactotroph, somatotroph)

Gastrinomas - duodenal

35
Q

3 MEN type 2A syndromes

A

Pheochromocytoma

Medullary thyroid carcinoma

Parathyroid hyperplasia (primary hyperparathyroidism)

36
Q

What mutation in seen in MEN 2A?

What mutation in seen in MEN 2B?

A

Germline GoF mutation in RET proto-oncogene

Germline GoF mutation in RET proto-oncogene (specific point mutation)

37
Q

3 MEN type 2B syndromes

A

Medullary thyroid carcinoma

Pheochromocytoma

Mucosal neuromas

38
Q

Which tumor can be part of MEN1 and MEN 2A?

A

Parathyroid adenoma (primary hyperparathyroidism)

39
Q

Which 2 tumors can be part of MEN2A and MEN2B?

A

Pheochromocytoma

Medullary thyroid carcinoma

40
Q

What are 4 “generalities” of MEN syndromes?

A

Patients develop tumors at a younger age

Tumors tend to be BL/multiple

Preceding hyperplasia is common

Tumors tend to be aggressive and recurrent

Endocrine & Reproduction II Exam #2 (10 decks)

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