Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Endocrine & Diabetes > Multiple Endocrine Neoplasia and Neuroendocrine Tumours > Flashcards

Multiple Endocrine Neoplasia and Neuroendocrine Tumours Flashcards

1
Q

How can a neuroendocrine tumour be differentiated from a metastatic tumour (stains)?

A
  • Chromogranin-A= blood tests
  • Synaptophysin= pathology use

=Grade / lymphovascular invasion etc etc
=Aka major synaptic vesicle protein p38 (SYP gene)
=Synaptic vesicle glycoprotein – present in neuroendocrine cells and brain and spinal cordalso present in the adrenal medulla (think phaeo etc)and pancreatic islet cells, small cell lung cancer and medullary thyroid carcinoma
=Hormone excess symptoms (flushing and diarrhoea)
= 24hr Urine 5-HIAA raised (downstream of serotonin pathway)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What does serotonin cause?

A
  • Flushing
  • Diarrhoea
  • Bronchospasm (can be hypotensive)
  • Right heart failure (serotonin to serotonin heart valves)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Why does right heart failure occur?

A

-Serotonin from neuroendocrine tumour cleared by enterohepatic circulation
-Too much= enterohepatic circulation overridden= hormones into IVC into right heart= heart valves
=Valve dysfunction (tricuspid regurgitation)
=Hepatic congestion (ascites)
=Peripheral oedema
=Elevated JVP
=Valve fibrosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What are the clinical characteristics of NETs?

A
  • Rare
  • Significant majority arise in GI system (including pancreas)
  • Usually slow growing= NETs or neuroendocrine carcinomas (G1-3)
  • Wide spectrum of disease activity
  • Often metastatic at presentation, late presentation
  • Prolonged survival is possible
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

How can patients present?

A
  • Enlargement of liver capsule= pain
  • Indigestion
  • Iron deficiency anaemia (GI cancer blood loss)
  • Appendicitis
  • Large tumour (terminal ileum common)= bowel obstruction, abdominal distention, vomiting and abdominal pain
  • Rectal= tenesmus, rectal bleeding
  • Pancreatic= CT imaging, abdominal pain, hormone presentation
  • Lung= cough, incidental finding, maybe thoracic pain
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What hormones are produced by neuroendocrine tumours and how to they present?

A
  • Glucagon= diabetes, hyperglycaemia, rash
  • Insulin= insulinoma= hypoglycaemia
  • Gastrin= heartburn and multiple peptide ulcers
  • Vasoactive intestinal polypeptide (VIP)= perfuse watery diarrhoea 10-15 times a day, hypokalaemia
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What are the malignant neuroendocrine tumours?

A

-Small cell lung cancer (poor prognosis)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What are the benign neuroendocrine tumours?

A
  • Appendiceal carcinoids
  • Insulinomas (good prognosis)
  • Gastric carcinoids
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What are the neuroendocrine tumours that are in-between benign and malignant?

A
  • Non-functioning pancreatic NETs
  • Gastrinomas
  • Glucagonomas
  • Small bowel carcinoids
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What are the functioning neuroendocrine tumours?

A

=produce hormones

  • Carcinoids
  • Gastrinomas
  • Glucagonomas
  • Insulinomas
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What are the non-functioning tumours?

A
  • Non-functioning NETS

- Bowel and pancreas

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What are the primary sites for neuroendocrine tumours?

A 
Mid-gut
=appendix (17%)
=ileum (16%)
=ileo-caecal junction (11%)
=caecum (3%)

Fore-gut
=stomach (7%)
=duodenum (4%)
=oesophagus (2%)

Hind-gut
=colorectal (7%)

Unknown (22%)- just finds metastases

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

How has the incidence of NETs changed?

A

-Much increased
=prevalence increased as people live longer
=more diagnosis as we are looking for them and are better at diagnosing

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

How is 5 year survival rates influenced by metastasis?

A

-Mets= 38%
-No nets= 85%
Overall 40%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What are the treatment options for NETs?

A
  • Active surveillance (no symptoms- side effects of treatments worse)
  • Surgery (bowel / pancreatic / hepatic)
  • Somatostatin analogue therapy= Lanreotide, expensive drug, octreotide
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What are the effects of somatostatin analogues?

A
  • Hormonal levels down for excess serotonin

- Anti-tumour effect= anti-proliferative, slow down rate of change

17
Q

Describe radionuclide therapy

A

-MIBG or radiolabelled somatostatin analogues (Lu)
-Must have positive uptake of relevant agent by neuroendocrine tumour
=periphery uptake + radiation crossfire
-Can target the relatively ischaemic central core of metastatic deposits
-Good for symptom control when SA no longer fully effective
-Potential toxicity to bone marrow and kidneys

18
Q

Describe transarterial chemoemolisation

A

=localised liver metastasis, deprive of blood supply as molecules block off vessel

  • Can be given in most large centres
  • Only targets cancer deposits in the liver
  • Destructive therapy so potential for rapid release of hormones from the dying cells= carcinoid crisis so lots of octreotide
  • This can cause major swings in blood pressure – in both the patient AND the interventional radiologist
19
Q

Describe multiple endocrine neoplasia

A

-Arises from tumour suppressor gene problem
=proliferation
-Relatively slow proliferation
=genetics, many screenings

20
Q

Describe the genetics of multiple endocrine neoplasia

A
  • 1:30,000

- Autosomal dominant inheritance, affects pretty much every generation

21
Q

What are the genetic defects in MEN1?

A
  • Defect in the MEN1 gene
  • Gene product is menin
  • Tumour suppressor gene
  • Chromosome 11
22
Q

What are the clinical features of MEN1?

A

3 Ps= pituitary, pancreas and parathyroid

  • Primary hyperparathyroidism
  • Pituitary adenomas
  • Pancreatic tumours (functional or non-functional)
  • Adrenal adenomas
  • Bronchial / Thymic carcinoids
  • (lipomas / angiofibroma)
23
Q

How do we screen for MEN1?

A

-Annual calcium and PTH
-Annual fasting gut hormones
=Chromogrannin A, insulin-glucose, gastrin glucagon, pancreatic polypeptide
-3 yearly MRI of pituitary and now pancreas
-Consideration for CT / MRI of chest and thymus

24
Q

What are the genetic defects in MEN2?

A
  • Defect in the MEN2 gene
  • Gene product is ret
  • Proto-oncogene gene
  • Chromosome 10
25
Q

What are the clinical features of MEN2?

A 
-2A (85%)
=Hyperparathyroidism
=Medullary thyroid cancer (prophylactic thyroidectomy)
=Phaeochromocytoma
-2B (5%)
=Hyperparathyroidism
=Medullary thyroid cancer
=Phaeochromocytoma
=neuromas, fibromas, musculoskeletal abnormalities
=Marfanoid habitus 
-Familial medullary thyroid cancer (15%)

Endocrine & Diabetes (45 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions