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7. Endocrinology (Medical Knowledge)a > Pathology of Endocrine System 2 > Flashcards

Pathology of Endocrine System 2 Flashcards

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USMLE® Step 1 flashcards
1
Q

Where is the pituitary gland located?

A

Sella turcica

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2
Q

What is the pituitary gland connected to the hypothalamus by?

A

Pituitary stalk

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3
Q

What are the 2 distinct components of the pituitary gland?

A

Anterior pituitary

Posterior pituitary

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4
Q

What is the anterior pituitary also called?

A

Adenohypophysis

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5
Q

Which componenet of the pituitary gland is the larger?

A

Anterior pituitary (is 80% of mass)

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6
Q

What does the anterior pituitary secrete?

A

ACTH

TSH

GH

Prolactin

FSH/LH

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7
Q

What is the secretion of hormones from the anterior pituitary controlled by?

A

Release factors from hypothalamus

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8
Q

Where does the blood supply to the anterior pituitary come from?

A

Hypothalamus

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9
Q

What is the posterior pituitary also called?

A

Neurohypophysis

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10
Q

What does the posterior pituitary secrete?

A

ADH

Oxytocin

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11
Q

What is the most common cause of pituitary hyperfunction?

A

Pituitary adenoma (carcinomas are very rare and also some hypothalamic disorders)

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12
Q

What age group does pituitary adenoma usually affect?

A

35-60 years

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13
Q

Is there a genetic predisposition to pituitary adenoma?

A

MEN-1

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14
Q

What is the effect of a functioning pituitary adenoma?

A

functioning = hormone excess

prolactinoma 20-30%

  • galactorrhoea, menstrual disorders

GH secreting

  • acromegaly, gigantism

ACTH secreting

  • cushing’s disease

non functioning (25-30%) = immunohistochemical demonstration

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15
Q

If a pituitary adenoma is a prolactinoma, what does it cause?

A

Galactorrhoea

Menstrual disorders

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16
Q

If a pituitary adenoma is a GH secreting, what does it cause?

A

Acromegaly

Gigantism

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17
Q

If a pituitary adenoma is a ACTH secreting what does it cause?

A

Cushing’s disease

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18
Q

What can happen if a pituitary adenoma is large?

A

if large -> mass pressure effect

  • radiographic abnormalities
  • visual field abnormalities
  • elevated intracranial pressure
  • compression damage, such as hypopituitarism
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19
Q

What percentage of pituitary function needs to be lost to be considered pituitary hypofunction?

A

75%

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20
Q

What can cause pituitary hypofunction?

A

Compression by tumours

Trauma

Infection (rare)

Post partum ischaemic necrosis

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21
Q

What infections can cause pituitary hypofunction?

A

TB

Sarcoidosis

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22
Q

What are the adrenal glands composed of?

A

Adrenal cortex

Adrenal medulla

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23
Q

What classification of hormones does the adrenal cortex secrete?

A

Steroid hormones

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24
Q

What are the 3 layers of the adrenal cortex?

A
  • Zona glomerulosa secretes mineralocorticoids (aldosterone)
  • Zona fasciculate secretes glucocorticoids (cortisol)
  • Zona reticularis secretes sex steroids
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25
Q

What does the zona glomerulosa secrete?

A

Minerlocorticoids (aldosterone)

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26
Q

What does the zona fasciculate secrete?

A

Glucocorticoids (cortisol)

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27
Q

What does the zona reticularis secrete?

A

Sex steroids

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28
Q

What does the adrenal medulla secrete?

A

Adrenaline/noradrenaline

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29
Q

What cells are found in the adrenal medulla?

A

Neuroendocrine (chromaffin) cells

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30
Q

What are some examples of the classifications of adrenal pathology?

A
  • hyperfunction
  • hypofunction
  • mass lesion
  • effect on adrenal gland: hyperplasia (diffuse or nodular), atrophy, mass lesion
31
Q

What are the 3 syndromes for adrenal cortical hyperfunction?

A

hypercortisolism -> cushing’s syndrome

hyperaldosteronism -> conn’s syndrome

adrenogenital syndromes

32
Q

What is hypercortisolism called?

A

Cushing’s syndrome

33
Q

What is hyperaldosteronism called?

A

Conn’s syndrome

34
Q

What is the aetiology of hypercortisolism?

A

exogenous

  • iatrogenic steroids

endogenous

  • ACTH dependent

> pituitary adenoma Cushing’s disease 70%

> ectopic ACTH

  • ACTH independent

> functioning adrenal adenoma 10%

35
Q

What is the main cause of ACTH dependent hypercortisolism?

A

Pituitary adenoma Cushing’s disease 70%

Ectopic ACTH

36
Q

What is the main cause of ACTH independent hypercortisolism?

A

Functioning adrenal adenoma

37
Q

How is hypercortisolism diagnosed?

A

Measuring cortisol, ACTH and response to ACTH suppresion

38
Q

What is the aetiology of hyperaldosteronism?

A

Bilateral idiopathic hyperplasia

Functioning adrenal adenoma

39
Q

What is the clinical presentation of hyperaldosteronism?

A

Increased sodium retention in nephron

Maybe asymptomatic

May have muscular weakness, tingling, spasms, hypertension, headaches

40
Q

What are some adrenogenital syndromes?

A
  • functioning adrenal tumour
  • pituitary tumour such as Cushing’s disease
  • congenital adrenal hyperplasia (due to steroid enzyme deficiency)
41
Q

What is the clinical presentation of adrenogenital syndrome?

A

Ambiguous genitalia

Precocious puberty

Failure of puberty

Infertility

42
Q

What is adrenal insufficiency caused by?

A

Destruction of the adrenal glands

43
Q

What can cause acute adrenal insufficiency?

A

Meningococal septicaemia

44
Q

What can cause chronic adrenal insufficiency?

A

Primary is Addison’s disease

  • autoimmune

> autoimmune polyendocrine syndromes

  • infections

> TB, fungus HIV related infections

  • replacement

> metastatic carcinoma amyloidosis

  • atrophy

> prolonged steroid therapy

  • congenital hypoplasia

Secondary is pituitary failure

45
Q

What are some causes of Addison’s disease?

A
  • Autoimmune
    • Autoimmune polyendocrine syndromes
  • Infections
    • TB, fungus HIV related infections
  • Replacement
    • Metastatic carcinoma amyloidosis
  • Atrophy
    • Prolonged steroid therapy
  • Congenital
46
Q

What do functioning adenomas in the adrenal cortex cause?

A

Hyperadrenal syndromes

Atrophy of adjacent cortex

47
Q

What is the histology of adrenal adenomas?

A
48
Q

What are the different kinds of adrenal carcinoma?

A

Primary adrenocortical carcinoma

Metastatic carcinoma

49
Q

Are primary adrenocortical carcinomas more likely to be functioning or non-functional?

A

Functional

50
Q

How does primary adrenocortical carcinoma often metastasis?

A

By lymphatics and blood, invades adrenal vein

51
Q

Is primary adrenocortical carcinoma or metastatic carcinoma of adrenal gland more common?

A

Metastatic carcinoma

52
Q

Where do metastatic carcinomas in the adrenal glands often come from?

A

Lung or breast

53
Q

What is an example of an adrenal medullary tumour?

A

Phaeochromocytoma

54
Q

What does phaeochromocytoma secrete and in turn cause?

A

Catecholamines causing hypertension

55
Q

Is there a genetic predisposition to phaeochromocytoma?

A

Up to 30% is inherited, such as due to MEN2 or SDH

56
Q

What is the histology of phaechromocytoma?

A

Nests of polygonal cells in vascular network

Granular cytoplasm containing catecholamines

57
Q

What are multiple endocrine neoplasia?

A

Inherited disorders with underlying gentic mutation

58
Q

What does MEN stand for?

A

Multiple endocrine neoplasia

59
Q

What does MEN cause?

A

Hyperplasia/neoplasms of endocrine organs

60
Q

When is the term MEN used?

A

Whe there is two or more distinct endocrine tumours due to one syndrome

61
Q

What are some different MEN syndromes?

A

MEN1 (Wermer syndrome)

MEN2 (MEN2A and MEN2B)

62
Q

What is MEN1 also known as?

A

Wermer syndrome

63
Q

What does the MEN1 tumour suppressor gene mutation cause defect in?

A

Menin protein

64
Q

What tumours are associated with MEN1 syndrome?

A
  • parathyroid hyperplasia and adenomas
  • pancreatic and duodenal endocrine tumours (causing hypoglycaemia and ulcers)
  • pituitary adenoma (prolactinoma)
65
Q

What is the function of the menin protein?

A

Involved in regulating cell growth

66
Q

What is MEN2 syndrome caused by?

A

RET proto-oncogene mutations

67
Q

What tumours are associated with MEN2?

A
  • medullary carcinoma of thyroid
  • phaeochromocytoma
68
Q

What is MEN2A also known as?

A

Sipple syndrome

69
Q

What is MEN2A due to?

A

Extracellular domain auto dimerisation of RET receptor

70
Q

What tumours are associated with MEN2A?

A

Medullary carcinoma of thyroid, phaeochromocytoma and parathyroid hyperplasia

71
Q

Is MEN2A or MEN2B more likely to affect younger patients?

A

MEN2B

72
Q

What is MEN2B due to?

A

Autoactivation of tyrosine kinase pathway

73
Q

What tumours are associated with MEN2B?

A
  • Causes medullary carcinoma of thyroid, phaeochromocytoma and parathyroid hyperplasia
  • Plus neuromas of skin and mucous membrane, skeletal abnormalities
74
Q

What is the normal function of RET gene?

A

Provide instruction for producing a protein involved in signalling within cells

7. Endocrinology (Medical Knowledge)a (28 decks)

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