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Clinical Pathology (RIP) > T3 - L1 Diseases of the endocrine system > Flashcards

T3 - L1 Diseases of the endocrine system Flashcards

1
Q

What is the difference between endocrine, paracrine and autocrine?

A

endocrine = secrete into blood stream and act systemically

paracrine = act locally

autocrine = affects the cell secreting the protein

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2
Q

Where is the pituitary gland situated?

A

in the sella turcica beneath the hypothalamus

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3
Q

What are the anterior and posterior parts of the pituitary also called?

A

anterior lobe (75%) = adenohypophysis (formed by outpouching of oral cavity)

posterior lobe (25%) = neuropophysis (formed by downgrowth of hypothalamus)

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4
Q

What are the causes of anterior pituitary hypofunction?

A
  • tumours (non-secretory adenoma, metastatic carcinoma)
  • trauma
  • infection
  • inflammation (granulomatous, autoimmune, other infections)
  • iatrogenic
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5
Q

Describe the features of primary pituitary tumours

A
  • vast majority are adenomas and benign
  • may be derived from any hormone-producing cell
  • effect hormone being produced
  • local effects due to pressure on optic chiasma or adjacent pituitary
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6
Q

Name the 3 types of anterior pituitary adenoma

A
  • prolactinoma
  • growth hormone secreting
  • ACTH secreting
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7
Q

What is the commonest anterior pituitary adenoma?

A

Prolactinoma

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8
Q

What do you get in prolactinoma pituitary adenomas?

A

Galactorrhoea and menstrual disturbance

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9
Q

What is galactorrhoea?

A

Excessive or inappropriate production of milk

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10
Q

What do you get in growth-hormone secreting pituitary adenomas?

A

Gigantism in children, acromegaly in adults

NB: caused by an increased production of growth hormone/ somatotropin

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11
Q

Describe the anatomy of the thyroid

A
  • Bilobed
  • joined by isthmus
  • encased in thin fibrous capsule
  • located at level 5th/6th/7th vertebra
  • anterior neck, close to trachea
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12
Q

Describe the anatomic relationships of the thyroid

A
  • abuts thyroid cartilage of larynx

- recurrent laryngeal nerve located in tracheo-oesophgeal groove, close to posterior aspects of lateral lobes

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13
Q

What is cretinism?

A

Severely stunted physical and mental growth due to untreated congenital deficiency of thyroid hormones (congenital hypothyroidism) usually due to maternal hypothyroidism

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14
Q

What is the most common type of thyroid ectopia?

A

Lingual thyroid (usually at base of tongue)

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15
Q

What occurs in most patients with lingual thyroid?

A
  • over 75% have no other thyroid tissue
  • 70% have hypothyroid
  • 10% have cretinism
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16
Q

Other than lingual thyroid, what are the other possible sites of ectopic thyroid?

A
  • sella turcica
  • larynx
  • trachea
  • aortic arch
  • oesophagus
  • heart
  • pericardium
  • liver
  • gall bladder
  • pancreas
  • vagina
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17
Q

Why do thyroglossal duct cysts occur?

A

Persistent track representing the embryological migratory path of thyroid anlage in the anterior neck

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18
Q

Who do thyroglossal duct cysts occur in?

A
  • most common in children and young adults

- 7% of adults

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19
Q

What is the treatment for thyroglossal duct cysts?

A

Sistrunk procedure (with 4-6% recurrence rate)

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20
Q

Where are the majority (75%) of thyroglossal duct cysts?

A

Anterior midline of neck or immediately below hyoid bone

- asymptomatic midline neck mass

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21
Q

What is acute thyroiditis?

A

Acute inflammation of the thyroid parenchyma associated with local or systemic viral, bacterial or fungal infection

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22
Q

Most cases of acute thyroiditis are due to what?

A

Generalised sepsis

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23
Q

What are the symptoms of acute thyroiditis?

A
  • fever
  • chills
  • malaise
  • pain
  • swelling of anterior neck
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24
Q

What does the prognosis of acute thyroiditis relate to?

A

The underlying condition

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25
Q

What is palpation thyroiditis?

A

Microscopic granulomatous foci centres on thyroid follicles - secondary to rupture of thyroid follicles due to palpation or surgery

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26
Q

Patients with palpation thyroiditis almost always have what?

A

A thyroid nodule

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27
Q

What is Riedel’s thyroiditis?

A

A rare fibrosing form of chronic thyroiditis

  • benign self-limited disease
  • may be mistaken for malignant neoplasm
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28
Q

In Riedel’s thyroiditis, the fibrosing disorder may also affect where?

A
  • retroperitoneum
  • lung
  • mediastinum
  • biliary tree
  • pancreas
  • kidney
  • subcutis
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29
Q

How may patients with Reidel thyroiditis present?

A
  • firm goitre
  • dysphagia
  • hoarseness
  • stridor
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30
Q

What is Hashimoto’s?

A

Chronic lymphocytic thyroiditis - autoimmune chronic inflammatory disorder associated with diffuse enlargement and thyroid autoantibodies

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31
Q

Who does Hashimoto’s occur in?

A
  • females much more common than males

- peak age at 59 years

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32
Q

What do you get in Hashimoto’s?

A
  • diffuse enlargement and thyroid autoantibodies.
  • serum thyroid antibodies elevated
  • lymphocytic infiltration of thyroid parenchyma
  • germinal centre formation
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33
Q

Is Hashimoto’s associated with hypothyroid or hyperthyroid?

A

Hypothyroid

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34
Q

What do patients with Hashimoto’s (chronic lymphocytic thyroiditis) have an increased risk of?

A
  • 80-fold increased risk of thyroid lymphoma

- increased risk of papillary carcinoma of the thyroid

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35
Q

What is Grave’s disease? (diffuse hyperplasia)

A

An autoimmune process results in clinical hyperthyroidism and diffuse hyperplasia of the
follicular epithelium.

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36
Q

What are the signs and symptoms of Grave’s disease? (diffuse hyperplasia)

A
  • hyperthyroidism
  • Pretibial myxoedema
  • hair loss
  • wide-eyed stare or proptosis
  • tachycardia
  • hyperactive reflexes.
  • thyroid diffusely enlarged.
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37
Q

What are the hormone changes in Grave’s disease (diffuse hyperplasia)?

A
  • T3 and T4 elevated

- TSH suppressed

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38
Q

What are the autoantibodies in Grave’s disease?

A

Thyroid stimulating immunoglobulins

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39
Q

What is a multinodular goitre?

A

Enlargement of thyroid with varying degrees of modularity

- 1 or more thyroid nodules discovered by patient or doctor

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40
Q

Most patient with multi nodular goitre are euthyroid. What does this mean?

A

Have a normally functioning thyroid gland

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41
Q

What symptom may occur with multi nodular goitre?

A
  • tracheal compression or dysphasia may occur with large nodules
  • dominant nodule may be mistaken clinically for thyroid carcinoma
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42
Q

What is a follicular adenoma?

A

A benign encapsulated tumour with evidence of follicular cell differentiation

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43
Q

is Grave’s disease more common in males or females?

A
  • females more than males
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44
Q

How does follicular adenoma present?

A
  • painless neck mass, often present for years
  • solitary nodule involving only one lobe
  • usually cold nodule on radioactive iodine imaging
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45
Q

What is the commonest type of papillary carcinoma?

A

Papillary carcinoma (over 70%)

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46
Q

Who does papillary carcinoma affect?

A

Females 2.5:1

  • mean 43 years
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47
Q

What is a papillary carcinoma?

A

A familial, autosomal dominant non-medullary thyroid carcinoma

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48
Q

Papillary carcinoma occurs as a result of what?

A
  • radiation exposure
  • FAP
  • Cowden’s syndrome
  • therapeutic irradiation
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49
Q

What genetic alterations cause papillary carcinoma?

A
  • activation of RET or NTRK1
  • RAS mutations
  • BRAF V600E mutation
  • variety of chromosomal translocations or inversions
  • fusion of RET tyrosine kinase regions with constitutively expressed thyroid proteins eg. PTC1 in inv(10)(q11;q21), PTC2 t(10;17)(q11;q21)
50
Q

Describe the macroscopic appearance of papillary carcinoma

A
  • ill-defined, infiltrative
  • some encapsulated
  • may be cystic
  • granular
51
Q

What is hurthle cell carcinoma also referred to as?

A

oxyphilic cell carcinoma

52
Q

What is primary hyperparathyroidism?

A

Excessive secretion of parathyroid hormone from one or more glands (PHPT)

53
Q

What is secondary hyperparathyroidism?

A

Hyperplasia of glands with elevated PTH in response to hypocalcaemia (due to renal insufficiency, malabsorption, vitamin D deficiency etc)

54
Q

What is tertiary hyperparathyroidism?

A

Adenoma in association with longstanding secondary hyperparathyroidism

55
Q

What are the potential symptoms/complications of PHPT?

A
  • asymptomatic
  • arterial hypertension
  • psychiatric problems
  • hypercalcaemia
  • decreased renal function
  • osteoporosis
  • hyperparathyroid bone disease
  • urolithiasis
56
Q

What are the 3 types of PHPT?

A
  • single adenoma (most)
  • diffuse chief/clear cell hyperplasia
  • carcinoma (1%)
57
Q

What is parathyroid adenoma?

A

An encapsulated benign neoplasm of parathyroid cells

Single enlarged parathyroid gland; remaining glands suppressed and small.

58
Q

The symptoms of parathyroid adenoma are due to what?

A

Hypercalcaemia

59
Q

Parathyroid adenoma is associated with which syndromes?

A
  • MEN1 and MEN2 syndrome
  • hyperparathyroidism
  • jaw tumour syndrome
60
Q

secondary and tertiary parathyroidism is a non-neoplastic increase in which cell mass?

A

parathyroid parenchymal cell mass within all parathyroid tissue

61
Q

Which group of patients is secondary and territory parathyroidism common in?

A

Patients with renal failure and on dialysis

62
Q

Secondary and territory parathyroidism have identical pathologic features to what?

A

Primary hyperplasia

  • may be associated with massive gland enlargement
63
Q

What cells does a parathyroid carcinoma derive from?

A

parathyroid parenchymal cells

64
Q

How is parathyroid carcinoma treated?

A

Surgery

65
Q

What is the prognosis for parathyroid carcinoma?

A

50% 10 year survival

66
Q

The symptoms of parathyroid carcinoma are referable to what?

A

Excess calcium

  • indolent with recurrence in about 50%
67
Q

What are the layers of the adrenal glands from the centre of the gland outwards?

A
  • medulla
  • reticularis (part of cortex)
  • fasciculata (part of cortex)
  • glomerulosa (part of cortex)
68
Q

What do the adrenal glands secrete?

A
  • catecholamines
  • androgens
  • cortisol
  • aldosterone
69
Q

What is Addison’s disease?

A

Primary adrenal cortical insufficiency

70
Q

What are the causes of Addison’s disease?

A
  • adrenal dysgenesis
  • adrenal dysfunction
  • autoimmune adrenalitis
  • TB
71
Q

What are the clinical features of Addison’s disease?

A
  • hyperpigmentation
  • postural hypotension
  • hyponatraemia
72
Q

How is Addison’s disease treated?

A

Long term steroid replacement

NB: high mortality if not diagnosed

73
Q

What are adrenal cortical nodules?

A

Benign non-functional nodules of adrenal cortex

74
Q

Adrenal cortical nodules occur more in who?

A
  • elderly
  • hypotensive
  • diabetic patients
75
Q

What are the symptoms of adrenal cortical nodules?

A
  • asymptomatic
  • incidental discovery on radiographic studies

NB: no treatment required

76
Q

What is adrenal cortical adenoma?

A

Benign neoplastic proliferation of adrenal cortical tissue

77
Q

What are the symptoms of adrenal cortical adenoma?

A

Symptoms related to endocrine hyperfunction (hypertension, Cushing’s syndrome, virilisation)

78
Q

What do aldosterone-producing tumours cause?

A

Conn’s syndrome

79
Q

What do cortisol-producing tumours cause?

A

Cushing’s syndrome

80
Q

What is Conn’s syndrome?

A

Excess production of the hormone aldosterone by the adrenal glands resulting in low renin levels

81
Q

Describe an adrenal cortical adenoma

A
  • unilateral solitary masses
  • 2cm, less than 100g
  • well-circumscribed yellow/brown nodules
  • formed from lipid-filled adrenal cortical cells
82
Q

What are the features of adrenal cortical carcinoma

A
  • malignant counterpart of adrenal adenoma
  • symptoms related to hormone excess
  • abdominal mass
  • prognosis is age and stage dependent
  • 5 year survival about 70%
83
Q

What is phaeochromocytoma?

A

Catecholamine-secreting tumour arising from adrenal medulla

84
Q

What would be elevated in a urine test for phaeochromocytoma?

A
  • urine catecholamine - adrenaline

- noradrenaline

85
Q

what familial syndromes can cause phaeochomocytoma?

A
  • MEN2a and 2b
  • von Recklinghausen’s disease
  • von Hippel-Lindau disease
86
Q

what clinical/functioning effects do primary pituitary tumours have?

A

effects the hormone that cell produces

87
Q

what local effects do primary pituitary tumours have?

A

pressure on optic chiasma or adjacent pituitary

88
Q

where does the anterior lobe of the pituitary gland derive from?

A

derived from an outpouching of the roof of the pharynx, called Rathke’s pouch

89
Q

what two hormones does the posterior pituitary gland produce?

A

ADH (responsible for control of blood osmolarity)

Oxytocin (involved in parturition and milk secretion)

90
Q

what is a prolactinoma?

A

a benign noncancerous tumor of the pituitary gland that produces a hormone called prolactin

91
Q

what is the hormone produced growth-hormone secreting pituitary adenomas called? and what does this result in?

A

somatotropin

  • giantism in children
  • acromegaly
92
Q

what is acromegaly?

A
  • Enlargement of the hands, feet, forehead, jaw, and nose, thicker skin, deepening of the voice
  • onset: middle age
  • cause: too much growth hormone/somatotropin
93
Q

what type of tumour would cause giantism in children?

A

Growth hormone secreting tumour of the pituitary gland

94
Q

what type of tumour would cause acromegaly in middle aged adults?

A

Growth hormone secreting tumour of the pituitary gland

95
Q

what is Cushing syndrome?

A
  • due to prolonged exposure to cortisol
  • high blood pressure
  • abdominal obesity but thin arms and legs
  • reddish stretch marks
  • fat lump between shoulders
  • weak muscles and bones
  • ache
  • fragile skin that heals poorly,
  • irregular menstuartion,
  • mood swings,
  • headaches
  • fatigue.
96
Q

what type of tumour would cause Cushing’s syndrome?

A

ACTH secreting tumour of the anterior pituitary gland

97
Q

what joins the two lobes of the thyroid?

A

isthmus

98
Q

what level vertebrae will you find the thyroid?

A

level of
5th, 6th and 7th vertebra

(between the C5 and T1 vertebrae)

99
Q

the thyroid becomes heavier during pregnancy, true or false?

A

true

average weight is 18g for adult males, 15g for adult females

100
Q

where will you find the pyramidal lobe of the thyroid?

A

on top of the isthmus

  • however it is a variant seen as a third thyroid lobe and is present in 10-30% of the population.
101
Q

which tracheal rings mark the location of the thyroid?

A

2nd and 3rd tracheal rings

102
Q

what nerves are commonly damaged during thyroid surgery?

A

recurrent laryngeal (left and right)

103
Q

where is the recurrent laryngeal nerve located in relation to the thyroid?

A

posteriorly of the tracheoesophageal groove

104
Q

what is the pyramidal lobe a remnant of?

A

It represents a persistent remnant of the thyroglossal duct

105
Q

what is the Ultimobranchial body?

A

In humans, the ultimobranchial body is an embryological structure that gives rise to the parafollicular cells of the thyroid gland.

106
Q

what is the most common type of thyroid carcinoma?

A

papillary carcinoma

107
Q

what hormone does the Glomerulosa layer of the adrenal gland produce?

A

Glomerulosa: outer layer producing aldosterone

108
Q

what are tumours arising from the glomerulosa layer of the adrenal gland called?

A

conn’s tumour/syndrome

109
Q

what do tumours of the glomerulosa layer of the adrenal gland layer (conn’s tumour) cause?

A

hyperaldosteronism

110
Q

what hormone does the fasciculata layer of the adrenal gland produce?

A

Fasciculata: thick middle layer producing cortisol

111
Q

Tumours arising from zona fasciculata are the most commonly cause what?

A

Cushing’s syndrome

112
Q

what hormone does the Reticularis layer of the adrenal gland produce?

A

Reticularis: inner layer producing androgens

113
Q

what hormone does the Adrenal medulla of the adrenal gland produce?

A

catecholamine

114
Q

what do medullary tumours cause?

A

increased serum catecholamine (Phaechromocytoma)

115
Q

what is increased serum catecholamine Phaechromocytoma?

A

Pheochromocytoma (PCC) is a neuroendocrine tumor of the medulla of the adrenal glands (originating in the chromaffin cells), or extra-adrenal chromaffin tissue that failed to involute after birth, that secretes high amounts of catecholamines

116
Q

what care exogenous causes of Cushing’s syndrome?

A

excessive glucocorticoid medication

117
Q

what care endogenous causes of Cushing’s syndrome?

A
  • adrenal cortical tumours
  • adrenal cortical hyperplasia
  • ACTH
    secreting pituitary adenoma
118
Q

what tumour is associated with Paraneoplastic cushing’s syndrome?

A

small cell lung carcinoma

rarer cause of Cushing’s

119
Q

what are symptoms of Cushing’s syndrome?

A
  • hypertension,
  • moon face,
  • abdominal obesity,
  • buffalo hump,
  • weak
    muscles,
  • osteoporosis,
  • insomnia,
  • excess sweating, - mood swings,
  • headaches,
  • chronic fatigue.
  • Women may have increased hair growth (hirsuitism) and irregular menstruation.
120
Q

excess production of what hormone is seen in Conn’s syndrome? what does this lead to?

A

aldosterone

leads to low renin levels

121
Q

what are signs and symptoms of conn’s syndrome?

A
  • high BP,
  • headache,
  • muscular weakness,
  • muscle spasms,
  • excessive
    urination,
  • cardiac arrythmias.

Clinical Pathology (RIP) (49 decks)

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