Neuroendocrine tumours Flashcards
(16 cards)
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)
what is a crinoid crisis and how can it prevented in surgery?
overwhelming release of hormones by the NET –> resistant severe hypotension
prevented by prophylactic octreotide (somatostatin analogue) before and after surgery
