Neuroendocrine tumours Flashcards by Sasha Murdoch

What do neuroendocrine cells contain

neurotransmitters
neuromodulators
neuropeptide hormones with secretory granules

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What are the differences between neurones and neuroendocrine cells

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

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What does the gastroenteropancreatic neuroendocrine system provide

the richest source of regulatory peptides outside the brain

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Where do neuroendocrine tumours originate from

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

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What are neuroendocrine tumours classified according to

Their embryological origin

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

whether a secreted hormone is detectable and associated symptoms are present

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What are neuroendocrine tumours sometimes part of

familial syndromes such as MEN1 or MEN2

neurofibromatosis type 1

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What is the term carcinoid used for

NETs mostly derived from serotonin-producing enterochromaffin cells

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

tumour size,
local spread, 
vascular invasion, 
metastases 
nuclear atypic

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

MEN 1

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How might a gastroenteropancreatic tumour present

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

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What is carcinoid syndrome

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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What is carcinoid syndrome characterised by

flushing
diarrhoea
occasionally wheezing

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What is pellagra

niacin deficiency

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What is pellagra characterised by

dermatitis
glossitis
diarrhoea
dementia

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Why might muscle wasting occur

as a result of poor protein synthesis

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What is carcinoid heart disease characterised by

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

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What is a carcinoid crisis characterised by

progound flushing
bronchospasm
achycardia
fluctuating blood pressure

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What might precipitate the carcinoid crisis

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

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What are the symptoms of insulinoma

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

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What are the symptoms of gastronome

severe peptic ulceration and diarrhoea

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What are the symptoms of glucagonoma

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

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What are symptoms of VIPoma (Wener-Morrison syndrome)

profuse watery diarrhoea with marked hypokalaemia

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What are symptoms of stomatostatinoma

cholelithiasis
weight loss
diarrhoea
steatorrhoea
diabetes mellitus

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What should patients with NETs be searched for

MEN 1 MEN 2 and NG 1

What is the gold standard investigation for gastroenteropancreatic NETs

detailed histology

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

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

What factors can affect urinary 5-hydrozyindoleacetic acid excretion

``` bananas avocados aubergines pineapples plums walnuts caffeine paracetamol naproxen ```

What is chromogranin A

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

What is the main metabolite of serotoning secretion

5-HIAA

What should be measured prior to giving glucose in suspected insulinoma

Insulin and C-peptide levels

What is diagnostic of an insulinoma

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

What are the investigations for a suspected gastrinoma

fasting gastrin level and gastric secretion studies

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

PPI (2 weeks) | histamine 2 blockers (3 days)

What is the most sensitive modality in assessing secondaries

SRS (somatostatin receptor scintigraphy)

What does treatment fr NETs depend on

symptoms stage of disease degree of uptake of radionuclide histological features

What is the curative treatment for NETs

surgery

How is a potential carcinoid crisis prevented

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

How do somatostatin analogues work in NETs

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

What are some side effects of somatostatin analogues

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

What are some side effects of alpha-interferon

``` Flu like symptoms weit loss fatigue depression hepatotoxicity autoimmune disorders ```

Who is hepatic artery embolisation indicated for

patients with non-resectable Multiple or hormone secreting tumours

How does artery embolisation work

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

What is the most common side effect of artery embolisation

post embolisation syndrome (nausea, fever, abdominal pain)

What is the overall 5 year survival of all NET cases

67%

What is MEN characterised by

tumours involving two or more endocrine glands within a gins patient

How are MEN syndromes inherited

autosomal dominant

What is MEN type 1 characterised by

predisposition to: parathyroid adenomas enteropancreatic endocrine adenomas pituitary adenomas

What do parathyroid adenomas result in

primary hyperparathyroidism and hypercalcaemia

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

prolactinomas

What does MEN1 encode for

protein called renin

What does renin do

suppresses gene transcription activated by JunD and control cells proliferation

What is found in 95% of patients with MEN1

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

What biochemical eating should be done for MEN1

serum calcium prolactin measurement of gut hormones

What is the management of MEN1

subtotal parathyroidectomy for hyperparathyroidism | surgical resection of pancreatic NETs

What does MEN2 result from

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

what endocrine tumours are associated with MEN 2

Medullary thyroid cancer (MTC) phaeochromocytoma parathyroid adenomas

What is the ideal treatment of MEN 2

Total thyroidectomy

Describe the differences in hyperparathyroidism in MEN1 and MEN2

It is milder in MEN2 | More than 1 parathyroid gland is enlarged in MEN2

What is MEN2

association of medullary thyroid cancer (MTC) with pheochromocytoma

Neuroendocrine tumours Flashcards

(60 cards)