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Lecture Notes - Endocrinology > Neuroendocrine tumours > Flashcards

Neuroendocrine tumours Flashcards

1
Q

What do neuroendocrine cells contain

A

neurotransmitters
neuromodulators
neuropeptide hormones with secretory granules

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2
Q

What are the differences between neurones and neuroendocrine cells

A

Neuroendocrine cells do not have axons and don’t make synapses

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3
Q

What does the gastroenteropancreatic neuroendocrine system provide

A

the richest source of regulatory peptides outside the brain

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4
Q

Where do neuroendocrine tumours originate from

A

neuroendocrine cells within the gut (75%)
pancreatic islet cells (5%)
lungs (15%)
Other organs)

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5
Q

What are neuroendocrine tumours classified according to

A

Their embryological origin

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6
Q

What determines whether a neuroendocrine tumour is functioning or non-functioning

A

whether a secreted hormone is detectable and associated symptoms are present

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7
Q

What are neuroendocrine tumours sometimes part of

A

familial syndromes such as MEN1 or MEN2

neurofibromatosis type 1

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8
Q

What is the term carcinoid used for

A

NETs mostly derived from serotonin-producing enterochromaffin cells

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9
Q

What does the classification of NETs into benign and malignant depend on

A 
tumour size,
local spread, 
vascular invasion, 
metastases 
nuclear atypic
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10
Q

Mutations in what gene are the most common form of genetic predisposition to NETs

A

MEN 1

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11
Q

How might a gastroenteropancreatic tumour present

A

may be asymptomatic or may present with obstructive symptoms due to tumour bulk (pain, nausea, vomiting)

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12
Q

What is carcinoid syndrome

A

it is usually a result of metastases to the liver with the subsequent release of hormones (serotonin, tachykinins and other vasoactive compounds) into the systemic circulaiton

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13
Q

What is carcinoid syndrome characterised by

A

flushing
diarrhoea
occasionally wheezing

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14
Q

What is pellagra

A

niacin deficiency

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15
Q

What is pellagra characterised by

A

dermatitis
glossitis
diarrhoea
dementia

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16
Q

Why might muscle wasting occur

A

as a result of poor protein synthesis

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17
Q

What is carcinoid heart disease characterised by

A

deposits of fibrous tissue on the endocardium of the valvular cusps and cardiac chambers

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18
Q

What is a carcinoid crisis characterised by

A

progound flushing
bronchospasm
achycardia
fluctuating blood pressure

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19
Q

What might precipitate the carcinoid crisis

A

anaesthetic induction
intraoperative handling of the tumour
invasive therapeutic procedures such as embolisation and radio frequency ablation

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20
Q

What are the symptoms of insulinoma

A

hypoglycaemia: sweating, dizziness, tachycardia, weakness, confusion, unconsciousness
symptoms relieved on eating

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21
Q

What are the symptoms of gastronome

A

severe peptic ulceration and diarrhoea

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22
Q

What are the symptoms of glucagonoma

A

necrolytic migratory erythema (rash affecting the lower abdomen, buttocks, perineum and groin
Weight loss
diabetes mellitus
stomatitis

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23
Q

What are symptoms of VIPoma (Wener-Morrison syndrome)

A

profuse watery diarrhoea with marked hypokalaemia

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24
Q

What are symptoms of stomatostatinoma

A 
cholelithiasis
weight loss
diarrhoea
steatorrhoea
diabetes mellitus
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25
Q

What should patients with NETs be searched for

A

MEN 1
MEN 2 and
NG 1

26
Q

What is the gold standard investigation for gastroenteropancreatic NETs

A

detailed histology

27
Q

What biochemical tests should be done for patients with symptoms suspicious of gastroenteropancreatic NET

A

Plasma chromogranin A
24 hour urinary 5- hydroxyindoleacetic acid
fasting gut hormone profile

28
Q

What factors can affect urinary 5-hydrozyindoleacetic acid excretion

A 
bananas 
avocados 
aubergines 
pineapples
plums 
walnuts 
caffeine 
paracetamol
naproxen
29
Q

What is chromogranin A

A

a large protein that is produced by all cells deriving from the neural crest

30
Q

What is the main metabolite of serotoning secretion

A

5-HIAA

31
Q

What should be measured prior to giving glucose in suspected insulinoma

A

Insulin and C-peptide levels

32
Q

What is diagnostic of an insulinoma

A

elevated plasma insulin and c peptide levels in the presence of hypoglycaemia
Plasma glucose of less than 2.2

33
Q

What are the investigations for a suspected gastrinoma

A

fasting gastrin level and gastric secretion studies

34
Q

What must the patient not be taking in order to accurately measure gastrin in a patient with a suspected gastronome

A

PPI (2 weeks)

histamine 2 blockers (3 days)

35
Q

What is the most sensitive modality in assessing secondaries

A

SRS (somatostatin receptor scintigraphy)

36
Q

What does treatment fr NETs depend on

A

symptoms
stage of disease
degree of uptake of radionuclide
histological features

37
Q

What is the curative treatment for NETs

A

surgery

38
Q

How is a potential carcinoid crisis prevented

A

IV infusion of octreotide at a dose of 50g per hour for 12 hours prior to and at least 48 hours after surgery

39
Q

How do somatostatin analogues work in NETs

A

They inhibit the release of various peptide hormones in the gut and antagonise growth factor effects on tumour cells

40
Q

What are some side effects of somatostatin analogues

A 
gall stones 
gall bladder dysfunction
fat malabsorption 
vitamin A and D malabsorption
headaches 
diarrhoea 
dizziness 
hyperglycaemia
41
Q

What are some side effects of alpha-interferon

A 
Flu like symptoms 
weit loss 
fatigue 
depression 
hepatotoxicity 
autoimmune disorders
42
Q

Who is hepatic artery embolisation indicated for

A

patients with non-resectable Multiple or hormone secreting tumours

43
Q

How does artery embolisation work

A

It induces ischaemia of the tumour cells thereby reducing their hormone output and causing liquefaction

44
Q

What is the most common side effect of artery embolisation

A

post embolisation syndrome (nausea, fever, abdominal pain)

45
Q

What is the overall 5 year survival of all NET cases

A

67%

46
Q

What is MEN characterised by

A

tumours involving two or more endocrine glands within a gins patient

47
Q

How are MEN syndromes inherited

A

autosomal dominant

48
Q

What is MEN type 1 characterised by

A

predisposition to:
parathyroid adenomas
enteropancreatic endocrine adenomas
pituitary adenomas

49
Q

What do parathyroid adenomas result in

A

primary hyperparathyroidism and hypercalcaemia

50
Q

What are the majority of pituitary adenomas that occur in MEN1 patients

A

prolactinomas

51
Q

What does MEN1 encode for

A

protein called renin

52
Q

What does renin do

A

suppresses gene transcription activated by JunD and control cells proliferation

53
Q

What is found in 95% of patients with MEN1

A

germline inactivating mutations in the tumour suppressor gene MEN 1 (chromosome 11)

54
Q

What biochemical eating should be done for MEN1

A

serum calcium
prolactin
measurement of gut hormones

55
Q

What is the management of MEN1

A

subtotal parathyroidectomy for hyperparathyroidism

surgical resection of pancreatic NETs

56
Q

What does MEN2 result from

A

germline mutations in the RET protooncogene (chromosome 10) which encodes a tyrosine kinase receptor

57
Q

what endocrine tumours are associated with MEN 2

A

Medullary thyroid cancer (MTC)
phaeochromocytoma
parathyroid adenomas

58
Q

What is the ideal treatment of MEN 2

A

Total thyroidectomy

59
Q

Describe the differences in hyperparathyroidism in MEN1 and MEN2

A

It is milder in MEN2

More than 1 parathyroid gland is enlarged in MEN2

60
Q

What is MEN2

A

association of medullary thyroid cancer (MTC) with pheochromocytoma

Lecture Notes - Endocrinology (40 decks)

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