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Robbins Pathology- Endocrine (Chapter 24) > Chapter 24- Adrenal, MEN, And Pineal > Flashcards

Chapter 24- Adrenal, MEN, And Pineal Flashcards

1
Q

What is the most common cause of cushing syndrome

A

Cushing disease (aka primary pituitary adenoma)

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2
Q

What are the tumors that can cause primary adrenal disease

A

10% adenoma

5% carcinoma

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3
Q

What is the size of the adrenal gland in the case of ectopic sources of cortisol and steroids

A

Atrophy

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4
Q

What is the size of the adrenal gland in the cause of ACTH dependant Cushing syndrome

A

Large

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5
Q

What is the difference between an adrenal cortical adenoma and carcinoma

A

The size, with greater than 200 grams being a carcinoma

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6
Q

What is Conn’s syndrome

A

Primary hyperaldosteronsim

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7
Q

What are the findings in Conn’s syndrome

A

aka Primary hyperaldosteronism

  • Hypertension
  • Hypokalemia
  • Hypomagnesemia
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8
Q

What is the reason for hypertension in Conn’s syndrome

A

Aka hyperaldosteronism

-Refractory hypertension with increased sodium and water uptake in the kidney

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9
Q

What are some of the common causes for secondary hyperaldosteronism

A
  • Diuretic use
  • Decreased renal perfusion
  • Arterial hypovolemic
  • Pregnancy
  • Renin secreting tumors
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10
Q

Which patients are seen to have aldosterone secreting adenomas

A

-Young Patients 30-40s

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11
Q

What are the size of aldosterone secreting adenoma

A

Small <2 cm

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12
Q

What are the histological findings in the case of aldosterone secreting adenoma

A

Spironolactone bodies

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13
Q

What are patients with aldosterone secreting adenomas at a higher risk of developing

A

-Ischemic heart disease

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14
Q

What should you investigate as the cause of high levels of plasma renin and aldosterone

A

Secondary hyperaldosteronism

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15
Q

What should you investigate as the cause of low levels of renin and aldosterone concentrations

A

Hyperaldosterone mimickers

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16
Q

What should you investigate as the cause of low levels of renin, but high levels of aldosterone

A

Primary hyperaldosteronism

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17
Q

What is the method of a pituitary causing virilization

A

ACTH stimulation of androgens

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18
Q

What are the adrenal causes of virilization

A
  • Primary adrenal neoplasms (adenoma and carcinoma)

- Congenital adrenal hyperplasia (CAH)

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19
Q

What is the most common adrenal neoplasm that causes virilization

A

Carcinoma

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20
Q

What is the inheritance of congenital adrenal hyperplasia (CAH)

A

-Autosomal recessive

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21
Q

What is the cause of congenital adrenal hyperplasia

A

Defective enzyme in the steroidogensis

  • 95% is caused by lack of 21-hydroxylase
  • Causes lack of mineral and clucocorticoids, so just all androgens
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22
Q

What is the presentation of congenital adrenal hyperplasia

A
  • Lack of 21 hydroxylase
  • Salt wasting ( no mineralcoriticoiuds) resulting in hyponatremia, hyperkalemia, and hypotension

-Virilization (at birth) in workmen

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23
Q

What is the long term effect of adrenomedullary dysplasia

A

Hypotension (Lack of aldosterone)

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24
Q

What is a method for testing for congenital adrenal hyperplasia

A
  • serum 17-hyroxyprogesterone levels

- ACTH stimulation test, as increasing ATCH will result in more androgens and no other product

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25
Q

What is the therapy for congenital adrenal hyperplasia

A

Glucocorticoids to replenish cortisol

Mineralcorticoid as necessary

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26
Q

What are the two very very general causes of primary adrenocortical insufficiency

A
  • Loss of cortical cells

- Metabolic failure in hormone production

27
Q

What are the some of the causes of cortical cell loss that leads to primary insufficiency

A
  • Autoimmune
  • infection (TB, Fungi)
  • Waterhouse friderichsen (adrenal hemorrhage)
  • Amyloidosis, sarcoidosis, hemochromatosis
  • Metastatic carcinoma
28
Q

What are the some of the causes of metabolic failure in hormone production that leads to primary adrenalcorticol insufficiency

A
  • Congenital adrenal hyperplasia (CAH)

- Drug or steroid induced inhibition of ACTH or cortisol cell function

29
Q

What is Waterhouse Friderichsen syndrome

A

-Adrenal hemorrhage, usually resulting in sepsis

30
Q

What are the signs of acute adrenal insufficiency

A
  • Hypotension
  • Abdominal Pain
  • Fever
  • Nausea/Vomiting
  • Hyponatremia
  • Hypoglycemia
31
Q

What are the signs of primary chronic adrenocortical insufficiency

A
  • Long duration of malaise, fatigue
  • Anorexia and weight loss
  • Joint pain
  • Hyperpigmentation of skin
32
Q

What is the infection can can commonly cause primary chronic adrenocortical insufficiency worldwide

A

TB

33
Q

What is the most common cause of primary chronic adrenocortical insufficiency in developed countries

A

Autoimmune

34
Q

Autoimmune polyendocrine syndrome type 1 is due to which mutation

A

AIRE

35
Q

What are the typical endocrine glands that are affected in Autoimmune polyendocrine syndrome type 1

A
  • Adrenalitis (usually the cause of death)
  • Parathyroiditis (usually the first presenting)
  • hypogonadism
  • pernicious anemia
36
Q

What are the other effects of Autoimmune polyendocrine syndrome type 1 commonly seen

A

Ectodermal dystrophy

-Mucocutaneous candiadisis (Abs against IL-17 and IL-22)

37
Q

What are the clinical presentations in someone with autoimmune polyendocrine syndrome type 1

A
  • Pointed teeth or baby teeth look
  • brittle hair
  • lack of ability to sweat
  • salivary secretions decreased
  • Nail beds may be lacking

*Also lack of PTH and with hypoaldrenalism

38
Q

What are the presentations in a patient with Autoimmune polyendocrine syndrome type 2

A
  • Adrenalitis
  • Thyrpiditis
  • T1DM
39
Q

What is the relation of adrenals to metastasis

A

Metastasis is commonly see in bilateral adrenals because they are so highly vascularized. Therefore they are common sites for metastasis to end up

40
Q

What is the cause to TB leading to chronic adrenal insufficiency

A
  • TB causes caseating granulomas in the adrenal gland

- Adrenal gland has necrosis and fibrosis and loss of function

41
Q

What type of chronic adrenal insufficiency is likely to be present if there are lymphocytic infilitration

A

Autoimmune

42
Q

What is the difference between cortical adenomas and carcinomas

A

The size, with adenomas being smaller and carcinomas being larger with the ability to compress adjacent structures

***Carcinomas can also cause virilization with increased production of androgens

43
Q

What is the cell type that makes up the adrenal medulla

A

Chromaffin cells from NC cells

44
Q

What are the products of the adrenal medulla and which brand of the ANS is controlling it

A

Secretes Epi and Norepinephrine

*Under SNS control

45
Q

What are the rule of 10’s in a phepchromocytoma

A

10% are:

  • Extra adrenal (paraglanlioma)
  • Bilateral
  • Kids
  • Malignant
  • Not associated with hypertension
46
Q

What percentage of pheochromocytomas are familial

A

25%

47
Q

What factor is seen in >90% of pheochromocytomas

A

Hypertension

48
Q

What is the classic triad for pheochromocytomas

A
  • Headache
  • Palpitations
  • Diaphoresis (sweating)
49
Q

In those patients with chronic pheochromocytomas, what is the complication seen

A

Cardiomyopathy

50
Q

What is the method used to diagnosis pheochromocytomas

A

Urine and plasma metanephrines

51
Q

What is the prognosis of an adrenal myelolopomas

A

Benign (just fat and bone marrow)

52
Q

How can a myelolipoma commonly present

A

Hemorrhage

53
Q

What is the testing done for adrenal incidentalomas

A

Postive functional assays:

  • Dethamexasone suppression test for hypercorticolism
  • Pheochromocytomas
54
Q

What is the gene mutated in MEN 1

A

Tumor suppressor gene Menin

55
Q

What are the neoplasms seen in MEN 1

A
  • Parathyroid (primary hyperPTH)
  • Pancreatic (usually insulinomas or gastrinomas)
  • Pituitary (adenomas-lactotroph and somatotrophs)
56
Q

What is the gene mutated in MEN 2A

A

RET gain of function in proto-oncogene

57
Q

What are the neoplasms seen in MEN 2A

A
  • Pheochromocytomas
  • Medullary thyroid carcinooma
  • Parathyroid hyperplasia
58
Q

What are the neoplasms seen in MEN 2B

A
  • Pheochromocytomas
  • Medullary thyroid carcinomas
  • Mucosal neuromas
59
Q

What conditions is commonly seen in MEN2B that is not part of the normal neoplasms seen

A

Marfan like syndrome in almost 100% of pts

60
Q

What is the gene mutated in familial medullary thyroid carcinoma

A

RET gain of function Proto-oncogene

61
Q

What tends to be the prognosis of MEN syndromes

A

Not good, as they tend to be aggressive and recurrent

62
Q

What are the generalities of MEN syndromes

A
  • Tumors in younger patients
  • Bilateral and multiple
  • Hyperplasia precedes
63
Q

What are the tumors seen in the pineal gland

A
  • germ cell tumors
  • Pineocytoma
  • Pineoblastoma

Robbins Pathology- Endocrine (Chapter 24) (5 decks)

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