Neuroendocrine tumours Flashcards
what are neuroendocrine cells?
these are cells that receive input from neurotransmitters to release hormones
where can Gastroenterioancreatic neuroendocrine tumors and what is another name for them?
- pancreas
- GI tract
also called carcinoid tumours when occurring in the GI tract
what are the main risk factors of GEP-NETs?
genetic:
- MEN1
- von Hipple-lindau disease (VHL)
- neurofibromatosis 1 (NF-1)
where do GEP-NETs occur most often?
appendix
what are the clinical features of GEP-NET?
- features of bowel obstruction
- non-specific symptoms (Abdo pain, N&V and distension)
- unintentional weight loss
- may go on to develop carcinoid syndrome
are the majority of GEP-NETs functioning or non-functioning?
- usually non-functioning
explain what carcinoid syndrome is and when does it occur?
metastasis of well-differentiated GEP-NETs (typically liver) –> sells begin to over secrete bioactive mediators e.g serotonin, prostaglandins and gastrin –> symptoms
- flushing (worse with alcohol/coffee)
- Abdo pain
- diarrhoea
- wheezing
- palpitations
what is the classification for grading GEP-NETs?
the WHO classification for GEP-NETs
this includes grades 1 - 3 (depending on differentiation on mitotic activity)
what investigations are performed in suspected GEP-NETS?
what are the relevant findings?
lab tests
- chromogranin A
- 5-HIAA (a metabolite of serotonin)
(pancreatic peptide and chromogranin B in pancreatic NETs)
specialized tests
with +ve GEP-NET bloods need imaging to assess mass size and location
endoscopy –> gastric, duodenal and colorectal NETs
CT enteroclysis –> for small bowel
genetic testing
how do you establish the extent of disease in metastatic disease?
whole body somatostatin receptor scintigraphy (SSRS)
what is the curative treatment for GEP-NETs?
surgery is the only curative treatment however patients
often present late with metastatic disease therefore surgery is often palliative
how do you treat gastric NETs?
type 1/2 –> endoscopic resection and annual surveillance (low metastatic potential)
type 3 –> partial/total gatrectomy with lymph node clearance (more aggresive lesions)
how do you treat small intestine NETs?
- resection of tumour with mesenteric lymph nodes regardless of liver metastases
- these tumours are always malignant
how do you treat appendiceal NETs?
depends on size :
> 2cm = appendicectomy and right hemicolectomy
< 2cm = appendicectomy
how do you treat colorectal NETs?
colonic: partial colectomy and regional lymph node clearance
* have the worst prognosis*
rectal = benign therefore can be treated with endoscopic resection (larger requiring AP/anterior resection)
