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Endocrinology > Neuroendocrine tumours > Flashcards

Neuroendocrine tumours Flashcards

1
Q

What is a neuroendocrine tumour (NET)?

A

A tumour that develops in the cells of the neuroendocrine system

They are usually cancerous

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2
Q

What is the neuroendocrine system?

A

A system of nerves and gland cells

The cells make hormones and release them into the blood stream

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3
Q

Where are neuroendocrine cells found in the body?

A

Most organs, particularly the

  • lungs
  • oesophagus
  • liver
  • stomach
  • small + large bowel
  • appendix
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4
Q

What do neuroendocrine cells do in the gut?

A

Release hormones that control release of digestive juices into the gut, and control the muscles to move food through the bowel

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5
Q

What do neuroendocrine cells do in the lungs?

A

Release hormones that control bronchoconstriction and dilation and vasoconstriction and dilation

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6
Q

Neuroendocrine tumours grow quickly. True or false?

A

False, they usually grow slowly

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7
Q

How do you classify neuroendocrine tumours?

A

If they are functioning and non-functioning

By where they are

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8
Q

What is a carcinoid tumour?

A

The old name for what is now neuroendocrine tumours

Carcinoid tumours now are defined as neuroendocrine tumours arising from the fore, mid and hindgut

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9
Q

List some types of neuroendocrine tumours of the pancreas?

A

Insulinoma: produce insulin

Gastrinoma: produce gastrin

Glucagonoma: produce glucagon

VIPoma: produce vasoactive intestinal peptide

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10
Q

Describe the classification of NETs by location?

A

They are either:

  1. Pancreatic endocrine tumours

OR

  1. Arising from foregut, midgut and hindgut (carcinoid)
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11
Q

Causes of NETs?

A

Some genetic syndromes cause NETs

We don’t know of any environmental causes

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12
Q

NETs (carcinoid tumours) arise from the fore, mid and hindgut. Name the most common places these tumours are seen in each of these?

A

Foregut: bronchus, stomach

Midgut: appendix, small bowel

Hindgut: rectum, colon

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13
Q

How are NETs usually discovered?

A

Incidentally

Or by presentation with carcinoid syndrome

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14
Q

What is carcinoid syndrome?

A

Caused by hormone secretion from the NET

Abdominal pain
Dry flushing of face (i.e. no sweating)
Diarrhoea often 10-20 times a day

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15
Q

NET liver metastases often have already developed before people present with carcinoid syndrome.
True or false?

A

True

Which is problematic

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16
Q

What causes death in carcinoid tumours?

A

Carcinoid heart disease: fibrotic heart valves

Cachexia

Liver failure (less often)

17
Q

Investigation of NETs?

A

5HIAA in urine is raised in 24 hour urine test

Chromogranin A

Imaging to find where it is, how big, any metastasis

Endoscopic USS

18
Q

What is 5HIAA and what is its relevance in NETs?

A

Tryptophan is broken down to make 5HIAA, it can be measured in urine

5HIAA is increased in NETs because they release chemicals that break it down?

19
Q

Treatment of NETs?

A

Treat the symptoms and the excessive hormone state

Curative surgery by removing tumour or debulking it

Treat metastatic disease

20
Q

Which hormones do NETs often produce too much of?

A

Somatostatin

21
Q

What drugs are used to treat NETs, specifically carcinoid tumours?

A

Somatostatin analogue therapy

Drugs which bind to receptors and switch of somatostatin production

Octreotide, lanreotide

22
Q

Name the main somatostatin analogue drugs? How are they administered?

A

Octreotide
Lanreotide

Injection

23
Q

What drugs are used to treat pancreatic endocrine tumours?

A

Everolimus

Sunitinib

24
Q

What are endocrine cancer syndromes?

A

Genetic conditions which cause high chance of developing neuroendocrine tumours

25
Q

Name 3 endocrine cancer syndromes?

A

MEN 1
MEN 2
Von Hippel Lindau

26
Q

Inheritance pattern of MEN 1 and 2?

A

Autosomal dominant

27
Q

Describe how MEN 1 causes endocrine cancers?

A

Mutation of one copy of the tumour suppressor gene

So if one gets silenced or damaged in life, they will have no tumour suppression, so will develop cancers

28
Q

What does MEN 1 cause in the body?

A

The three Ps

Pituitary tumours
Parathyroid tumours
Pancreatic tumours

29
Q

When and how do patients with MEN 1 present?

A

The parathyroid is often affected first

Causes excess parathyroid hormone, which causes calcium to break out of the bones and into the blood stream

They often present with bone problems in their 20s

30
Q

What does ‘MEN’ stand for?

A

Multiple endocrine neoplasia

31
Q

What does MEN 2 cause in the body? There are 2 types!

A

Type A

  • medullary thyroid cancer
  • hyperparathyroidism
  • Phaeochromocytoma

Type B

  • all of above
  • ganglioneuroma
  • marfanoid habitus
32
Q

At what age do patients with MEN 2 present?

A

MEN 2 causes cancers very early

Children with known family history of MEN 2 are screened at an early age

If they have MEN 2 they undergo a thyroidectomy as prophylaxis

33
Q

What is Von Hippel Lindau?

A

Autosomal dominant condition of the VHL gene which causes increased development of:

  • Phaeochromocytoma
  • Renal cell carcinoma
  • CNS haemangioma affecting retina, spinal cord, cerebellum

Endocrinology (19 decks)

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