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Endocrine- MHD > Adrenal Gland > Flashcards

Adrenal Gland Flashcards

1
Q

From what germ layer is the adrenal cortex derived?

A

Mesoderm

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2
Q

From what tissue does the adrenal medulla arise?

A

Neuroectoderm

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3
Q

What is secreted by the zona glomerulosa?

A

Mineralocorticoids- aldosterone

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4
Q

What is secreted by the zona fasciculata?

A

Glucocorticoids- cortisol

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5
Q

What is secreted by the zona reticularis?

A

Sex steroids- progestins, estrogens, androgens

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6
Q

What is the most common cause of Cushing’s syndrome?

A

Exogenous administration of glucocorticoids (corticosteroids)

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7
Q

What is Cushing’s disease?

A

Subset of Cushing’s syndrome:

Hypercortisolism caused by primary hypothalamic pituitary disease associated with hypersecretion of ACTH.

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8
Q

What is the most common cause of Cushing’s disease?

A

Pituitary adenoma leading to excess secretion of ACTH

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9
Q

Most of the time, are adrenal adenomas functional or non-functional?

A

Non-Functional!

If they are functional, they produce the mineralocorticoids or glucocorticoids

Generally, functional carcinomas or hyperplasia leads to secretion of sex hormones

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10
Q

What is Conn Syndrome?

A

Aldosterone producing adenoma of the adrenal gland

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11
Q

What is the most common cause of hyperaldosteronism?

A

Bilateral idiopathic hyperaldosteronism- nodular hyperplasia of the adrenal glands

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12
Q

What are the clinical manifestations of hyperaldosteronism?

A

Hypertension and hypokalemia

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13
Q

Describe the pathology of congenital adrenal hyperplasia

A

AR enzyme deficiency in the synthesis of cortisol leads to excess ACTH and consequently adrenal hyperplasia. Excess precursors build up and this leads to increased production of androgens.

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14
Q

Which is the most common enzyme deficiency leading to congenital adrenal hyperplasia?

A

21 hydroxylase

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15
Q

Why must withdrawal from corticosteroid therapy be tapered?

A

Acute withdrawal can lead to adrenal insufficiency

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16
Q

What is Addison’s Disease?

A

Destruction of the adrenal cortex with multiple etiologies leading to hypofunction

17
Q

What part of the adrenal glands are destroyed in autoimmune adrenalitis?

A

All cortical zones

18
Q

Name two inherited disorders that predispose you to an adrenal carcinoma

A

Li-Fraumeni - germline mutations in p53

Beckwith Wiedemann Syndrome- (overgrowth disorder)

19
Q

What is the prognosis of adrenal carcinomas?

A

Very bad- survival less than 2 years

They have a tendency to invade the vasculature

20
Q

What is the cell of origin of a pheochromocytoma?

A

Chromaffin cells (neuroendocrine cells) of the adrenal medulla

21
Q

What is the main symptoms of a pheochromocytoma?

A

HTN- due to release of catecholamines

22
Q

Name 6 genes associated with familial pheochromocytomas

A

RET (Men II and III)
NF1
VHL
SDHB, SDHC, SDHD

23
Q

What is required for the definition of malignant pheochromocytoma?

A

Metastasis

24
Q

Labile or paroxysmal hypertension is associated with which adrenal pathology?

A

Pheochromocytoma

25
Q

Where do neuroblastomas arise?

A

In the adrenal medulla or extra-adrenal paraganglionic tissues

26
Q

How do neuroblastomas appear on histology?

A

Small, round blue cells arranged in rosettes

27
Q

What is the inheritance pattern of MEN I?

A

Autosomal dominent

28
Q

What is the gene affected in MEN I?

A

MENI gene: 11q13 - a tumor suppressor gene

29
Q

What are the three organs involved in MEN I?

A

3 Ps

Pancreas, parathyroid, and pituitary

30
Q

What is the most common manifestation of MEN I?

A

Primary hyperparathyroidism- hyperplasia or adenoma

31
Q

What is the leading cause of death in MEN I?

A

Endocrine tumors of the pancreas

They are functional tumors and they metastasize

32
Q

What is the most commonly secreted hormone from the pituitary adenomas of MEN I

A

Prolactin

33
Q

What is the inheritance pattern of MEN 2A?

A

Autosomal Dominant

34
Q

What is the gene affected in MEN 2A?

A

RET protooncogene (10q11.2)

35
Q

What are the three organs affected in MEN 2A?

A

Thyroid- medullary carcinomas
Adrenal Medulla- pheochromocytomas
Parathyroid- hyperthyroidism

36
Q

What is the most common primary manifestation of MEN 2A?

A

Medullary carcinoma of the thyroid is seen in virtually all untreated cases in the first 2 decades

37
Q

What differentiates MEN 2A from MEN 2B?

A

No parathyroid manifestations, but instead there are mucosal ganglioneuromas with marfanoid habitus observed

38
Q

What is the recommendation for patients found to have Ret mutations?

A

Prophylactic thyroidectomy to prevent the onset of medullary carcinomas

Endocrine- MHD (17 decks)

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