Adrenal Gland Pathology Flashcards

1
Q

What is the adrenal cortex derived from?

A

Mesoderm

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2
Q

What is the adrenal medulla derived from?

A

Neuroectoderm

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3
Q

Cushing Syndrome (Hypercortisolism)

A

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

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4
Q

What is the most common cause of Cushing Syndrome?

A

Exogenous administration of glucocorticoids

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5
Q

Cushing’s Disease

A

Primary hypothalamic pituitary disease associated with the hypersecretion of ACTH

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6
Q

What gender is more affected by Cushing’s syndrome?

A

Males

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7
Q

What are the 4 main causes of Cushing syndrome?

A
  • Exogenous glucocorticoids
  • Pituitary ACTH oversecretion
  • Lung tumor paraneoplastic ACTH secretion
  • Adrenal cortisol producing tumor
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8
Q

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

A

Atrophy

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9
Q

What are the clinical features of Cushing syndrome?

A
  • HTN
  • Weight gian
  • Buffalo hump
  • Truncal obesity
  • Increased risk of infection
  • Bone resorption
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10
Q

What are the clinical manifestations of hyperaldosteronism?

A

HTN with hypokalemia and increased Na+ retention

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11
Q

What are some primary causes of hyperaldosteronism?

A
  • Familal genetic defect
  • Tumors
  • Idiopathic
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12
Q

Congenital Adrenal Hyperplasia

A

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

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13
Q

What is the most common defect in congenital adrenal hyperplasia?

A

21-alpha hydroxylase

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14
Q

Waterhouse-Friderichsen Syndrome

A

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

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15
Q

What is the most common cause of chronic adrenal insufficiency?

A

Autoimmune Adrenalitis

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16
Q

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

A
  • Septicemia
  • Hypotension
  • Massive bilateral adrenal hemorrhage
17
Q

Addison’s Disease

A

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

18
Q

What is hyperpigmentation a sign of?

A

Increased ACTH - decreased adrenal sufficency

19
Q

Adrenal Carcinoma

A

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

20
Q

What is the best criteria for malignancy in adrenocortical carcinoma?

A

Invasion

21
Q

Pheochromocytoma

A

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

22
Q

What is seen on histology of pheochromocytoma?

A

Zellballen (cellular balls or nests)

23
Q

What are the clinical features of pheochromocytoma?

A
  • HTN
  • Headache
  • Sweating
  • Tremor
  • Elevated VMA in urine
24
Q

Neuroblastoma

A

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

25
Q

MEN 1

A

Autosomal dominant

  • Pituitary adenoma
  • Parathyroid
  • Pancreatic tumors
26
Q

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

A

MEN1 - Ch 11 (tumor suppressor)

27
Q

MEN 2A

A

Autosomal Dominant

  • Medullary thyroid carcinoma
  • Pheochromocytoma
  • Parathyroid
28
Q

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

A

Ret - Ch 10 (protooncogene)

29
Q

MEN 2B

A

Autosomal Dominant

  • Medullary thyroid carcinoma
  • Pheochromocytoma
  • Mucosal neuromas
30
Q

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

A

Ret - Ch 10 (protooncogene)

31
Q

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

A

D. Many patients develop parathyroid hyperplasia