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Endocrine > Pathology of the Adrenal gland > Flashcards

Pathology of the Adrenal gland Flashcards

1
Q

What is the role of aldosterone?

A

Causes reabsorption of Na and water, and secretion of K

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2
Q

What is the function of the adrenal glands, in order?

A 
G= Aldosterone secretion
F= Cortisol
R= Sex hormones

“it gets sweeter as it gets deeper”

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3
Q

What are the two primary adrenal cortical neoplasms? Which is more common?

A
  • Cortical adenoma-more common

- Cortical carcinoma

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4
Q

What are the typical characteristics of an adrenal cortical adenoma? Where in the gland does it lie?

A

Well circumscribed, yellow-orange lesion that usually lies in the cortex or protrudes into the medulla or the subcapsular region

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5
Q

Why are adrenal cortical adenomas yellow-orange in color?

A

High lipid content

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6
Q

What is the genetic mutation that is affected with neuroblastomas?

A

n-Myc

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7
Q

Do adrenal adenomas lie within or outside the cortex?

A

May lie within, or protrude into medulla

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8
Q

What are the general characteristics of large (greater than 1 cm) adrenal cortical adenomas?

A

Areas of hemorrhage, cystic changes, and calcification

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9
Q

How can you differentiate between functional and nonfunctional adrenal adenomas?

A

Only through lab findings

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10
Q

Adenomas are distinguished from nodular hyperplasia how?

A

Solitary, circumscribed mass

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11
Q

What are the histological characteristics of adrenal cortical adenomas? (3)

A

Vacuolated d/t the presence of intracytoplasmic lipid

  • Mild nuclear pleomorphism
  • No mitotic activity
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12
Q

Why are adrenal cortical adenomas vacuolated?

A

d/t the presence of intracytoplasmic lipid

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13
Q

True or false: adrenal cortical carcinomas are not very malignant, relatively

A

False–very malignant

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14
Q

What are the characteristics of the cut surface of adrenocortical neoplasms?

A

Yellow on cut surface, but usually contain areas of hemorrhage, cystic changes, and necrosis

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15
Q

What are the histological characteristics of adrenocortical neoplasms?

A

Range from well differentiated to markedly anaplastic cells

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16
Q

Where do adrenal adrenocortical carcinomas usually metastasize to?

A

Lymph nodes viscera and lungs

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17
Q

What is the only way to differentiate between malignant adrenocortical neoplasms from benign ones?

A

If it spreads

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18
Q

What happens to the kidneys with adrenal carcinomas?

A

Compression

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19
Q

What are the four major characteristics of anaplastic cells in adrenocortical carcinomas?

A

1, Pleomorphic

  1. Abnormal nuclear morph
  2. Mitoses
  3. Loss of polarity
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20
Q

What does it mean for cells to be pleomorphic?

A

Different sizes and shapes

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21
Q

What are the characteristics of abnormal nuclear morphology of adrenal cortical carcinomas?

A

variable nuclear condensation

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22
Q

What characteristics, besides metastases, are found with adrenocortical carcinomas and not with adenomas?

A
  • Large size
  • Necrosis
  • Mitotic figures
  • Vascular invasion
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23
Q

What are two characteristics of adrenocortical neoplasms that 100% define them to be malignant?

A
  • Mets

- Vascular invasion

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24
Q

What are the three major characteristics of adrenal cortical hyperplasia (Color, thickness, nodularity)?

A

Yellow
Thickened
Multinodular

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25
Q

What is the difference between hypertrophy and hyperplasia?

A

Hypertrophy = increase in cell size

Hyperplasia = increase in number of cells

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26
Q

What are the three distinctive hyperadrenal syndromes?

A
  • Cushing’s syndrome
  • Hyperaldosteronism
  • Adrenogenital syndromes
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27
Q

What, generally, is Cushing’s syndrome?

A

Elevation in glucocorticoid levels

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28
Q

What is Cushing’s disease?

A

Pituitary adenoma causing an increase in ACTH secretion

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29
Q

What is Cushing’s syndrome?

A

Excess cortisol or ACTH for some reason besides a pituitary pathology

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30
Q

What are the four major physical manifestations of Cushing’s?

A
  • central obesity
  • Moon facies
  • Cutaneous striae
  • Hirsutism
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31
Q

Hypo or hypertension with Cushing’s?

A

HTN

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32
Q

True or false: menstrual abnormalities are common in Cushing’s

33
Q

True or false: neuropsych problem are common in Cushing’s

34
Q

What are the characteristics of the moon facies seen with Cushing’s?

A
  • Cannot see ears
  • Round face
  • Cannot see nose from side
35
Q

What is primary hyperaldosteronism?

A

-Aldosterone producing adrenocortical neoplasm, usually an adenoma
OR
-Primary adrenocortical hyperplasia

36
Q

What is Conn syndrome?

A

A solitary aldosterone secreting adenoma or the adrenal gland

37
Q

What are the characteristics of the cut surface of an aldosterone producing adenoma?

A

Bright yellow d/t high lipid content

38
Q

Are the adenomas seen in Conn’s syndrome usually solitary or diffuse? Small or large? Encapsulated or not?

A

Solitary
Small
Encapsulated

39
Q

What are the key lab values that are abnormal in hyperaldosteronism? (4)

A
  • Hypocalcemia
  • Hypokalemia
  • Low renin
  • High aldosterone
40
Q

What is the classical presentation of a pt with primary hyperaldosteronism?

A

Young person with resistant HTN

41
Q

Is renin usually low or high with hyperaldosteronism? Why?

A

Low, since HTN will increase delivery of solutes to the distal nephron

42
Q

What is the eponym for chronic primary adrenal insufficiency?

A

Addison’s’ disease

43
Q

What is the name for acute adrenocortical insufficiency?

A

Adrenal crisis

44
Q

What is Waterhouse-Friderichsen syndrome? What is the usual causative agent?

A

Adrenal gland failure due to hemorrhage into the adrenal glands, usually occurring after sepsis with neisseria meningitidis

45
Q

In whom does Waterhouse-Friderichsen syndrome usually occur in?

46
Q

In whom does Addison’s disease usually occur in? How much of the adrenal gland must be destroyed for this to occur?

A

Adults who suffer at least 90% destruction of their adrenal cortex

47
Q

True or false: Addison’s disease is an autoimmune condition

48
Q

What are the two infectious agents that classically lead to the development of Addison’s disease?

49
Q

Which carcinoma classically lead to the development of Addison’s disease?

A

Carcinomas of the lung and breast

50
Q

What are the three major histological characteristics of Addison’s disease?

A
  • Small glands
  • Lipid depletion of the adrenal cortex
  • Lymphocytic infiltration of the cortex
51
Q

Which part of the adrenal gland is usually spared with autoimmune adrenalitis?

A

Adrenal medulla

52
Q

A pt with a h/o autoimmunity presenting with hyperpigmentation should be suspicious for what?

A

Addison’s disease (primary adrenal insufficiency)

53
Q

What are the major electrolyte disturbances with Addison’s disease?

A

Hyperkalemia

Hyponatremia

54
Q

What are the general ssx of Addison’s?

A

n/v

  • Anorexia
  • Cutaneous hyperpigmentation
55
Q

HTN or hypotension with Addison’s disease? Why?

A

Hypotension d/t loss of aldosterone and cortisol

56
Q

What zones of the adrenal glands are spared with secondary adrenocortical insufficiency? What is the clinical significance of this?

A

Zons glomerulosa and medulla

This means that aldosterone production is unaffected, and thus there are no electrolyte disturbances or BP changes

57
Q

What are the general causes of secondary adrenocortical insufficiency?

A

Hypothalamic or pituitary disorder, leading to Decreased production of ACTH

58
Q

In what type of adrenocortical insufficiency is there hyperpigmentation, primary or secondary?

A

Primary d/t increased ACTH and POMC production

59
Q

What are the “five 10%” rules of pheochromocytoma?

A
  • 10% malignant
  • 10% nonfunctional
  • 10% bilateral
  • 10% extra adrenal
  • 10% familial
60
Q

What, generally, is the range of sizes of pheochromocytomas?

A

1 g - 4 kg

61
Q

What are the characteristics of the cut surface of a pheochromocytoma?

A

Gray or brown, and is often associated with hemorrhage, necrosis, or cystic changes

62
Q

True or false: pheochromocytomas are rarely vascular

A

False–highly

63
Q

What is the agent that is used to fix pheochromocytomas? What does this do?

A

Zenker

Causes it to turn brown-black d/t oxidation of catecholamines

64
Q

What are the two neuroendocrine markers for a pheochromocytoma?

A

Synaptophysin +

Chromogranin +

65
Q

What are the histological characteristics of a pheochromocytoma?

A

Mature, polygonal to spindle-shaped medullary cells with basophilic granules

66
Q

What are the reliable histologic predictors of malignancy for pheochromocytom

A

None–only mets can tell if malignant

67
Q

Where do pheochromocytomas usually metastasize to? (4)

A

Lymph nodes
Liver
Lungs
Bone

68
Q

What four other organ dysfunctions appear with pheochromocytomas?

A
  • CHF
  • MI
  • Arrhythmias
  • Cerebral hemorrhage
69
Q

How do you diagnose a pheochromocytoma?

A

Measure urinary and serum catecholamines and their metabolites

70
Q

What is the specific imaging modality for pheochromocytomas?

A

MIBG scan (I-131 will localize)

71
Q

What are neuroblastomas? Where anatomically do they usually occur? In whom are they seen?

A

Tumors derived from neural crest cells d/t an n-Myc amplicifcation.

Usually extracranial and in the adrenal medulla

Sporadically in children under 5 y.o.

72
Q

What are the histological characteristics of Neuroblastomas?

A

Small blue cell tumors with Homer-Wright rosettes

73
Q

What will a scanning EM show with neuroblastomas?

A

Neurosecretory granules

74
Q

What are the clinical PE findings with neuroblastomas?

A

Palpable abdominal mass

Diastolic HTN

75
Q

Where do neuroblastomas usually met to?

A

Skin and bones

76
Q

What are the markers for neuroblastomas?

A

Increased urinary VMA, metanephrines and HVA

77
Q

What are the cutaneous manifestations of neuroblastomas?

A

Blueberry muffin baby

78
Q

What are adrenal gland ganglioneuromas?

A

Tumor composed of mature ganglion cells and neuronal elements

79
Q

What is the mutation that causes Wilms tumor?

A

WT1 gene on chromosome 11

Endocrine (32 decks)

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