Neuroendocrine tumours (e.g. carcinoid tumors)

Question 1

Carcinoid tumors

Answer 1

enterochromaffin cells (serotonin producing) of the intestine

Question 2

Carcinoid syndrome

Answer 2

the symptoms that arise as a result of serotonin (5-hydroxytryptamine, 5-HT), kinins, histamine and prostaglandins released into the circulation by the tumour

Question 3

Patients with carcinoid tumors only have carcinoid syndrome if they have ________

Answer 3

liver mets

Question 4

what clinical features do tumour products cause when they drain directly into the hepatic vein (without being metabolised by the liver) and into the systemic circulation?

Answer 4

flushing 
wheezing
diarrhoea
abd pain
right-sided cardiac valvular fibrosis --> stenosis and regurgitation

Question 5

What’s found in the urine in carcinoid syndrome?

Answer 5

High level of 5-hydroxyindoleacetic acid (5-HIAA), the breakdown product of serotonin

Question 6

How to confirm the presence of liver mets?

Answer 6

liver ultrasound

Question 7

How do you inhibit the tumour products?

Answer 7

somatostatin analogue, octreotide or with 5-HT antagonists e.g. cyproheptadine

Question 8

How do you reduce the tumour mass?

Answer 8

surgical resection
hepatic artery embolisation
radio frequency ablation
chemo

Question 9

is the Mx of carcinoid syndrome symptomatic?

Answer 9

YES

Question 10

Other types of NET (not carcinoid)

Answer 10

pancreatic neuroendocrine tumours (islet cell tumours:
non-functional; insulinomas; gastrinomas;
glucagonomas)

phaeochromocytomas

Question 11

What receptors do carcinoid tumours usually express?

Answer 11

somatostatin receptors