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

Chapter 24- Adrenal, MEN, And Pineal Flashcards

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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
What is the therapy for congenital adrenal hyperplasia
Glucocorticoids to replenish cortisol | Mineralcorticoid as necessary
26
What are the two very very general causes of primary adrenocortical insufficiency
- Loss of cortical cells | - Metabolic failure in hormone production
27
What are the some of the causes of cortical cell loss that leads to primary insufficiency
- Autoimmune - infection (TB, Fungi) - Waterhouse friderichsen (adrenal hemorrhage) - Amyloidosis, sarcoidosis, hemochromatosis - Metastatic carcinoma
28
What are the some of the causes of metabolic failure in hormone production that leads to primary adrenalcorticol insufficiency
- Congenital adrenal hyperplasia (CAH) | - Drug or steroid induced inhibition of ACTH or cortisol cell function
29
What is Waterhouse Friderichsen syndrome
-Adrenal hemorrhage, usually resulting in sepsis
30
What are the signs of acute adrenal insufficiency
- Hypotension - Abdominal Pain - Fever - Nausea/Vomiting - Hyponatremia - Hypoglycemia
31
What are the signs of primary chronic adrenocortical insufficiency
- Long duration of malaise, fatigue - Anorexia and weight loss - Joint pain - Hyperpigmentation of skin
32
What is the infection can can commonly cause primary chronic adrenocortical insufficiency worldwide
TB
33
What is the most common cause of primary chronic adrenocortical insufficiency in developed countries
Autoimmune
34
Autoimmune polyendocrine syndrome type 1 is due to which mutation
AIRE
35
What are the typical endocrine glands that are affected in Autoimmune polyendocrine syndrome type 1
- Adrenalitis (usually the cause of death) - Parathyroiditis (usually the first presenting) - hypogonadism - pernicious anemia
36
What are the other effects of Autoimmune polyendocrine syndrome type 1 commonly seen
Ectodermal dystrophy | -Mucocutaneous candiadisis (Abs against IL-17 and IL-22)
37
What are the clinical presentations in someone with autoimmune polyendocrine syndrome type 1
- 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
What are the presentations in a patient with Autoimmune polyendocrine syndrome type 2
- Adrenalitis - Thyrpiditis - T1DM
39
What is the relation of adrenals to metastasis
Metastasis is commonly see in bilateral adrenals because they are so highly vascularized. Therefore they are common sites for metastasis to end up
40
What is the cause to TB leading to chronic adrenal insufficiency
- TB causes caseating granulomas in the adrenal gland | - Adrenal gland has necrosis and fibrosis and loss of function
41
What type of chronic adrenal insufficiency is likely to be present if there are lymphocytic infilitration
Autoimmune
42
What is the difference between cortical adenomas and carcinomas
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
What is the cell type that makes up the adrenal medulla
Chromaffin cells from NC cells
44
What are the products of the adrenal medulla and which brand of the ANS is controlling it
Secretes Epi and Norepinephrine *Under SNS control
45
What are the rule of 10’s in a phepchromocytoma
10% are: - Extra adrenal (paraglanlioma) - Bilateral - Kids - Malignant - Not associated with hypertension
46
What percentage of pheochromocytomas are familial
25%
47
What factor is seen in >90% of pheochromocytomas
Hypertension
48
What is the classic triad for pheochromocytomas
- Headache - Palpitations - Diaphoresis (sweating)
49
In those patients with chronic pheochromocytomas, what is the complication seen
Cardiomyopathy
50
What is the method used to diagnosis pheochromocytomas
Urine and plasma metanephrines
51
What is the prognosis of an adrenal myelolopomas
Benign (just fat and bone marrow)
52
How can a myelolipoma commonly present
Hemorrhage
53
What is the testing done for adrenal incidentalomas
Postive functional assays: - Dethamexasone suppression test for hypercorticolism - Pheochromocytomas
54
What is the gene mutated in MEN 1
Tumor suppressor gene Menin
55
What are the neoplasms seen in MEN 1
- Parathyroid (primary hyperPTH) - Pancreatic (usually insulinomas or gastrinomas) - Pituitary (adenomas-lactotroph and somatotrophs)
56
What is the gene mutated in MEN 2A
RET gain of function in proto-oncogene
57
What are the neoplasms seen in MEN 2A
- Pheochromocytomas - Medullary thyroid carcinooma - Parathyroid hyperplasia
58
What are the neoplasms seen in MEN 2B
- Pheochromocytomas - Medullary thyroid carcinomas - Mucosal neuromas
59
What conditions is commonly seen in MEN2B that is not part of the normal neoplasms seen
Marfan like syndrome in almost 100% of pts
60
What is the gene mutated in familial medullary thyroid carcinoma
RET gain of function Proto-oncogene
61
What tends to be the prognosis of MEN syndromes
Not good, as they tend to be aggressive and recurrent
62
What are the generalities of MEN syndromes
- Tumors in younger patients - Bilateral and multiple - Hyperplasia precedes
63
What are the tumors seen in the pineal gland
- germ cell tumors - Pineocytoma - Pineoblastoma

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

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