Neuroendocrine Tumours

Question 1

What are gastroenteropancreatic Neuroendocrine tumours (GET-NETs )

Answer 1

Gastroenteropancreatic neuroendocrine tumours (GEP-NETs) refers to neuroendrocrine tumours originating from neuroendocrine cells in the tubular gastrointestinal tract and the pancreas

Question 2

What is the grading classification used for GEP NETs

Answer 2

WHO classification ranging from grade 1 - 3

Question 3

What is WHO grade 1 for NET

Answer 3

Well differentiated, mitotic count < 2/10 HPF

Question 4

What are the grade 2 WHO classification for NET

Answer 4

Well differentiated, mitotic count 2-20/10 HPF

Question 5

What is grade 3 WHO classification for NET

Answer 5

Poorly differentiated, mitotic count > 20 per 10 HPF

Question 6

What are the risk factors for GEP-NETs

Answer 6

Genetic disorders:

• MEN1
• Von Hippel Lindau
• neurofibromatosis
• tuberous sclerosis complex

Question 7

What are the clinical features of NETs

Answer 7

Vague abdo pain 
N+V
Abdo distention 
Bowel obstruction 
Unintentional weight loss

Question 8

What is carcinoid syndrome?

Answer 8

Carcinoid syndrome occurs following metastasis of a carcinoid tumour, whereby the metastasised cells begin to oversecrete bioactive mediators, such as serotonin, prostaglandins, and gastrin, into the circulation.

Question 9

What investigations would you carry out for suspected GEP-NET

Answer 9

Chromogranin A
5-HIAA 
FBC 
LFTs
Chromogranin B 
Pancreatic Peptide 

For other specific NET such as insulinomas, VIPomas, gastrinomas etc

Question 10

What is the best imaging modality to assess small bowel NET

Answer 10

CT enterolysis

Question 11

What is the only curative treatment option available for GEP NETs

Answer 11

Surgery

Question 12

What is carcinoid crisis

Answer 12

Carcinoid crisis is caused by an overwhelming release of hormones from the NET, resulting in a resistant severe hypotension.

Question 13

What drugs may be used for the prophylaxis of carcinoid crisis.

Answer 13

Somatostatin analogues may be used for the prophylaxis of carcinoid crisis

Question 14

What is the management of grade 1 and 2 gastric NETs

Answer 14

Type 1 and type 2 have a very low metastatic potential, therefore can usually be treated with endoscopic resection and annual surveillance

Question 15

What is the management of grade 3 Gastric NETs

Answer 15

Type 3 are more aggressive lesions, therefore management is often partial or total gastrectomy with lymph node clearance.

Question 16

How do you treat Small intestine NET

Answer 16

Resection of the tumor with mesenteric lymph node clearance regardless of liver mets

Question 17

How do you treat Appendiceal NETs

Answer 17

Appendiceal NETs have a more benign course than small intestinal NETs, however management depends on their size; tumours >2cm require appendicectomy with right hemicolectomy, whilst those <2cm may be treated with appendicectomy alone.

Question 18

How do you treat Colonic NETs

Answer 18

Colonic NETs have the worst prognosis of any gastrointestinal NET, with their treatment often requiring a partial colectomy and regional lymph node clearance.