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X - Cancer Care COPY > Neuroendocrine Tumours > Flashcards

Neuroendocrine Tumours Flashcards

1
Q

Do neuroendocrine tumours grow fast or slow?

A

Slow

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2
Q

What might neuroendocrine tumours secrete?

A

Bioactive peptides and hormones

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3
Q

Where can neuroendocrine tumours arise?

A

Anywhere in body, but most common in;

  • GI tract
  • Lungs
  • Pancreas
  • Pituitary
  • Parathyroid
  • Thyroid
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4
Q

Are neuroendocrine tumours usually malignant or benign?

A

Benign

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5
Q

What do the symptoms result from in neuroendocrine tumours?

A

Hormone production

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6
Q

What can happen to malignant neuroendocrine tumours?

A

Malignant transformation

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7
Q

What can NETs be classified based on?

A
  • Site of origin
  • Histological appearance
  • Embryological origin
  • Grade of tumour
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8
Q

What do functioning NETs do?

A

Produce bioactive substances that produce clinical symptoms

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9
Q

What do NETs arising from the GI tract require to produce symptoms?

A

Liver mets, to produce symptoms due to first-pass metabolism

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10
Q

Do non-functioning NETs secrete bioactive substances?

A

They may secrete bioactive substances, but often at subclinical levels

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11
Q

How do non-functioning NETs produce symptoms?

A

Most commonly due to physical effects of tumour

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12
Q

What is often true of NETs from a particular anatomical origin?

A

They often show similar behaviour as a group

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13
Q

Give 3 examples of anatomical origins

A
  • Foregut
  • Midgut
  • Hindgut
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14
Q

What arises from the foregut?

A
  • Oesophagus
  • Stomach
  • Duodenum
  • Liver
  • Pancreas
  • Thymus
  • Lung
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15
Q

What arises from the midgut?

A
  • Bowel from jejunum to transverse colon

- Appendix

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16
Q

What arises from the hindgut?

A
  • Descending colon
  • Sigmoid
  • Rectum
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17
Q

Are foregut NETs argentaffin positive or negative?

A

Negative

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18
Q

What hormones do foregut NETs often secrete?

A
  • 5-HTP
  • Histamine
  • Several polypeptide hormones
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19
Q

What might foregut NETs be associated with?

A
  • Atypical carcinoid syndrome
  • Acromegaly
  • Cushing’s disease
  • Other endocrine disorders
  • Telangiectasia
  • Hypertrophy of skin in face and upper neck
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20
Q

Where can foregut NETs metastasise too?

A

Bone

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21
Q

Are midgut NETs argentaffin positive or negative?

A

Positive

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22
Q

What hormones do midgut NETs secrete?

A
  • 5-HT (high levels)
  • Kinins
  • Prostaglandins
  • Substance P
  • Other vasoactive peptides
  • ACTH
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23
Q

Is bone mets common with midgut NETs?

A

No, uncommon

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24
Q

Are hindgut NETs argentaffin positive or negative?

A

Negative

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25
Q

What hormones do handout NETs rarely secrete?

A
  • 5-HT
  • 5-HTP
  • Any other vasoactive peptides

baso they don’t do much

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26
Q

Where do hindgut NETs often metastasise too?

A

Bone

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27
Q

What are carcinoid tumours derived from?

A

Enterochromaffin cells

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28
Q

What do carcinoid tumours usually release?

A

Serotonin

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29
Q

Where do carcinoid tumours most commonly arise?

A

Appendix and ileum

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30
Q

What % of carcinoid tumours arise in the appendix and ileum?

A

90%

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31
Q

What are carcinoid tumours associated with?

A

Carcinoid syndrome

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32
Q

What are the symptoms of carcinoid syndrome?

A
  • Flushing
  • Wheeze
  • Diarrhoea
  • Hypotension
  • Telangiectasia
  • Bronchoconstriction
  • Heart disease
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33
Q

What causes carcinoid syndrome in carcinoid tumours?

A

High serotonin levels

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34
Q

In what % of carcinoid tumours does carcinoid syndrome occur?

A

10%

35
Q

Why does carcinoid syndrome only occur in 10% of carcinoid tumours?

A

Because it requires liver metastases to bypass the effects of first pass metabolism

36
Q

What specific investigations should be done in carcinoid tumours?

A
  • 5-hydroxyindoleacetic acid in a 24-hour urine collection

- Plasma tachykinins

37
Q

What is the standard imaging investigation in carcinoid tumours?

A

Somatostatin receptor scintigraphy

38
Q

How is somatostatin receptor scintigraphy performed?

A

Radioisotope-labelled ocreotide is injected IV and binds with high affinity to somatostatin receptors 2 and 5, which are commonly found in NETs

39
Q

What does somatostatin receptor scintigraphy allow in carcinoid tumours?

A

Location of tumours to be visualised

40
Q

What is the primary management of carcinoid tumours?

A

Surgery

41
Q

When can surgery produce long remission periods in carcinoid tumours?

A

Following complete resection of the tumour and affect lymph nodes

42
Q

What % of patients with carcinoid tumours have significant improvement in symptoms following resection?

A

70-90%

43
Q

What do somatostatin analogues do in carcinoid tumours?

A
  • Control symptoms

- Prevent progression

44
Q

When are somatostatin analogues used in carcinoid tumours?

A

When complete resection is impossible or prior to surgical management

45
Q

How do somatostatin analogues work in carcinoid tumours?

A

They inhibit hormone secretion by binding to the somatostatin receptor subtypes loca located on neuroendocrine cells

46
Q

Is chemotherapy used in carcinoid tumours?

A

No significant effect in typical disease, but may be used in widely metastatic of high grade NETs, or those not responsive to conventional treatment

47
Q

What is radiotherapy used for in carcinoid tumours?

A

Targeted radiotherapy can be used to relieve pain from bone mets

48
Q

What are the types of gastroenteropancreatic NETs?

A
  • Insulinomas
  • Gastrinomas
  • Glucagonoma
  • VIPoma
  • Somatostatinoma
  • Pheochromocytoma
49
Q

What is an insulinoma?

A

Insulin secreting tumour

50
Q

What is an insulinoma compromised of?

A

Beta islet cells

51
Q

Are insulinomas malignant or benign?

A

Usually benign

52
Q

How does an insulinoma produce symptoms?

A

Normal feedback mechanisms for the maintenance of normoglycemia are ineffective, resulting in hypoglycaemia

53
Q

What symptoms are produced by hypoglycaemia in insulinomas?

A
  • Diplopia
  • Blurred vision
  • Confusion
  • Seizures
  • Coma
  • Weakness
  • Sweating
  • Tremor
  • Tachycardia
  • Palpitations
  • Anxiety
54
Q

What triad can be a feature of insulinomas?

A

Whipples triad

55
Q

What is Whipple’s triad?

A
  • Symptoms of hypoglycaemia
  • Hypoglycaemia measured during symptoms
  • Symptom relief following administration of glucose
56
Q

Why do you need to take a drug history in a patient presenting with suspected insulinoma?

A
  • Sulfonylureas stimulate insulin release

- Accidental administration of wrong insulin dose can produce these symptoms

57
Q

How can you differentiate between exogenous and endogenous insulin?

A

Endogenous insulin breaks down into insulin and C-peptide, but C-peptide is absent with exogenous insulin

58
Q

What are gastinomas?

A

Rare tumours associated with excess gastrin secretion

59
Q

What can excess gastrin secretion result in?

A

Gastric and duodenal ulceration

60
Q

Who should gastrinoma be suspected in?

A

Patients with multiple and atypical ulceration

61
Q

What are glucagonomas?

A

Tumours formed of alpha islet cells in the pancreas

62
Q

What do glucagonomas result in?

A

Increased plasma glucagon levels

63
Q

What are the clinical features of glucagonomas?

A
  • Diabetes
  • Dermatitis
  • DVT
  • Depression
64
Q

What do VIPomas secrete?

A

Vasoactive intestinal polypeptide (VIP)

65
Q

What does VIP do?

A

Stimulates secretion and inhibits reabsorption of water, potassium sodium, and chloride in the small bowel

66
Q

How do VIPomas present?

A
  • Dehydration
  • Hypokalaemia
  • Achlorhydria
  • High-volume odourless diarrhoea that persists despite fasting
67
Q

What is true of most VIPomas at the time of diagnosis?

A

They are metastatic

68
Q

How is a diagnosis of VIPoma made?

A

Fasting plasma VIP

69
Q

What does a somatostatinoma result in?

A

Inhibition of almost all gastric hormones

70
Q

Why do most patients with somatostatinomas present late?

A

Because the typical presentation is vague abdominal discomfort

71
Q

What symptoms may be present in somatostatinoma?

A
  • Gallstones
  • Steatorrhoea
  • Mild diabetes
  • Weight loss
  • Reduced stomach acid
72
Q

What do pheochromocytomas secrete?

A

Catecholamines

73
Q

Where do pheochromocytomas occur?

A

In the medulla of the adrenal glands

74
Q

What are pheochromocytoma associated with?

A

Multiple endocrine neoplasia 2 (MEN 2)

75
Q

How many pheochromocytomas present?

A
  • Incidentally

- WIth features of increased levels of circulating catecholeamines

76
Q

How might pheochromocytoma present incidentally?

A

With intermittent severe hypertension or unresponsive essential hypertension

77
Q

What symptoms might result from increased levels of circulating catecholeamines?

A
  • Headaches
  • Tachycardia
  • Palpitations
  • Sweating
  • Pallor
  • Tremor
78
Q

How is pheochromocytoma diagnosed?

A

Urine and plasma catecholamines

79
Q

What is usually required in the management of pheochromocytoma?

A

Antihypertensive therapy

80
Q

How do growth hormone secreting tumours present?

A
  • Acromegaly in adults, gigantism in chidlren
  • Coarse facial features
  • Excessive sweating
81
Q

How do gonadotrophin (LH/FSH) secreting tumours present?

A

Usually asymptomatic, however rarely have;

  • Testicular enlargement
  • Ovarian hyperstimulation
82
Q

How do prolactin secreting tumours present?

A
  • Amenorrhoea
  • Infertility
  • Reduced libido
  • Galactorrhoea
  • Hypogonadism
83
Q

How do thyroid hormone secreting tumours present?

A
  • Goitre
  • Tremors
  • Palpitations
  • Weight loss
  • Anxiety
84
Q

How do corticotrophin secreting tumours present?

A

Symptoms of Cushing’s syndrome;

  • Central obesity
  • Hyperglycaemia
  • Hisutism
  • Acne
  • Moon shaped face

X - Cancer Care COPY (36 decks)

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