Carcinoid syndrome

Question 1

Define Carcinoid syndrome

Answer 1

set of Signs and Sx caused from a carcinoid tumour secreting excess amounts of Serotonin (5HT) and other vasoactive peptides in circulation

many midgut endocrine tumours, bronchial and pancreatic endocrine tumours secrete serotonin

classification follows NET classification
For/Mid/Hind Gut
Typical-less argressive
Atypical-agressivr
functional vs non functional

Question 2

Aetiology and risk factors Carcinoid syndrome

Answer 2

Excess serotonin from neuroendocrine tumours, from gut diffuse endocrine cells-main cause
occasionally from bronchial neuroendocrine tumour, and pancreatic NET
about 30% of people with MIDgut NET and 5% with bronchial NET (1% Foregut/pancreas NET) get carcinoid
nearly always seen with liver metastases

Risk factors
Age-Older (60-70)
MEN-1 mutation are in 10% of NET, but rarely with carcinoid

Question 3

Epidiemology of Carcinoid syndrome

Answer 3

0.5 per 100 000 in Sweden
Arises from FUNCTIONAL (only about40% of NET) NET’s
Higher (3.8 / 100 00) in US
Most common functional NET

Question 4

Signs and Sx of Carcinoid syndrome

Answer 4

Typical is diarrhoea and flushing (with stress or tyramine containing food (cheese, wine) present years before diagnosis

Occasional palpitation and tachycardia

abdo cramps

Teleganctasia on cheeks

Cardiac murmurs from fibrosis of valves

Hepatomegaly from liver metastases (common in NET)

Question 5

Investigations of Carcinoid syndrome

Answer 5

Main test-
Serum Chromagranin A/B-specialised test for NET (secreted by NETS)

Urine serotonin break down (5-Hydroindoleacetic acid)

diarrhoea can cause dehydration (rise urea and creat)

FBC -fine
LFT-depends on metastases

CT to identify primary tumours and metastases

Question 6

mx of serotonin syndrome

Answer 6

octeroitide/somatostatin analogues
diarrhoea management