Adrenal Gland Pathology Flashcards Preview

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Flashcards in Adrenal Gland Pathology Deck (31):
1

What is the adrenal cortex derived from?

Mesoderm

2

What is the adrenal medulla derived from?

Neuroectoderm

3

Cushing Syndrome (Hypercortisolism)

Can be due to secretion of ACTH by tumors in pituitary or elsewhere leading to hypercortisolism with bilateral hyperplasia of adrenals

4

What is the most common cause of Cushing Syndrome?

Exogenous administration of glucocorticoids

5

Cushing's Disease

Primary hypothalamic pituitary disease associated with the hypersecretion of ACTH

6

What gender is more affected by Cushing's syndrome?

Males

7

What are the 4 main causes of Cushing syndrome?

- Exogenous glucocorticoids
- Pituitary ACTH oversecretion
- Lung tumor paraneoplastic ACTH secretion
- Adrenal cortisol producing tumor

8

What will happen to the adrenal glands with exogenous glucocorticoid administration?

Atrophy

9

What are the clinical features of Cushing syndrome?

- HTN
- Weight gian
- Buffalo hump
- Truncal obesity
- Increased risk of infection
- Bone resorption

10

What are the clinical manifestations of hyperaldosteronism?

HTN with hypokalemia and increased Na+ retention

11

What are some primary causes of hyperaldosteronism?

- Familal genetic defect
- Tumors
- Idiopathic

12

Congenital Adrenal Hyperplasia

Autosomal recessive with a defect in enzyme involved in adrenal steroid synthesis and decreased cortisol synthesis leads to increased androgen synthesis

13

What is the most common defect in congenital adrenal hyperplasia?

21-alpha hydroxylase

14

Waterhouse-Friderichsen Syndrome

Sudden withdrawal of long-term corticosteroid therapy leads to stress with underlying acute adrenal insufficiency

15

What is the most common cause of chronic adrenal insufficiency?

Autoimmune Adrenalitis

16

What are some of the causes of Waterhouse-Friderichson syndrome?

- Septicemia
- Hypotension
- Massive bilateral adrenal hemorrhage

17

Addison's Disease

Autoimmune adrenalitis that leads to atrophy of the glands with thinning of the cortices with the medulla often unaffected

18

What is hyperpigmentation a sign of?

Increased ACTH - decreased adrenal sufficency

19

Adrenal Carcinoma

Rare - some inherited cases like Li-Fraumeni syndrome and are quite invasive with poor survival

20

What is the best criteria for malignancy in adrenocortical carcinoma?

Invasion

21

Pheochromocytoma

A neoplasma arising from the chromaffin cells in the paraganglion system and can cause HTN

22

What is seen on histology of pheochromocytoma?

Zellballen (cellular balls or nests)

23

What are the clinical features of pheochromocytoma?

- HTN
- Headache
- Sweating
- Tremor
- Elevated VMA in urine

24

Neuroblastoma

Neoplasm arising in the adrenal medulla or paraganglion tissue that are large and bulky

25

MEN 1

Autosomal dominant
- Pituitary adenoma
- Parathyroid
- Pancreatic tumors

26

What gene is MEN I associated with and where is it found?

MEN1 - Ch 11 (tumor suppressor)

27

MEN 2A

Autosomal Dominant
- Medullary thyroid carcinoma
- Pheochromocytoma
- Parathyroid

28

What gene is MEN 2A associated with and where is it found?

Ret - Ch 10 (protooncogene)

29

MEN 2B

Autosomal Dominant
- Medullary thyroid carcinoma
- Pheochromocytoma
- Mucosal neuromas

30

What gene is MEN 2b associated with and where is it found?

Ret - Ch 10 (protooncogene)

31

Which of the following is true regarding multiple
endocrine neoplasia (MEN) I syndrome?
A. Virtually all patients develop medullary thyroid
carcinoma
B. The genetic abnormality involves the RET gene
C. The genetic abnormality involves a protooncogene
D. Many patients develop parathyroid hyperplasia
E. Some patients may have a Marfanoid habitus

D. Many patients develop parathyroid hyperplasia

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