European Neuroendocrine Tumor Society (ENETS) 2023 guidance paper for gastric neuroendocrine tumours (NETs) G1–G3

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The ENETS 2023 guideline for gNETs are combined with the guidelines for Duodenal NET (dNET) due to their close relationship in anatomical terms. 

Gastric neuroendocrine neoplasms (gNENs) are tumours with an increasing annual incidence and.  The vast majority of gNENs are well-differentiated neuroendrocrine tumours (NETs), which are usually classified according to the background gastric pathology into three major categories known as “types” – these should not be confused with “grades” which are generated by histopathological tissue sampling (biopsy). 

Type I when chronic atrophic gastritis (CAG) is present resulting in hypergastrinaemia (high Gastrin) and high gastric pH. They are the most common type, accounting for 75%–80% of all gNENs. Type I gNETs are normally ‘indolent’ with negligible risk of metastases (<5%) and excellent long-term survival (almost 100%). 

Type II when the tumour occurs due to hypergastrinaemia (high Gastrin) but low gastric pH and in the context of Zollinger–Ellison syndrome (ZES) via a Gastrinoma.  They most often associated with multiple endocrine neoplasia type I (MEN-I) syndrome.  Sporadic Type II is rare. This type accounts for 5% of gNENs.  

Type III, which are sporadic lesions not associated with hypergastrinaemia and with normal gastric pH (15%–25% of gNENs).  Type III tumours are usually more aggressive, owing to the high prevalence of metastatic disease (>50%) and the more unfavourable long-term survival (5-year survival rate 70%), although they present well-differentiated morphology in the majority of cases. Type III gNENs may also have higher grades. 

TABLE 1. Seven clinical questions for gNET scenarios.

(The reference document covers Gastric NETs (1-7) and Duodenal NETs (8-10))

Q1 – What is the role of endoscopic resection for type I gNETs? Is additional treatment required in cases of R1 endoscopic resection?
Q2 – When should somatostatin analogues be used to treat type I gNETs?
Q3 – What is the recommended follow-up schedule in type I gNETs?
Q4 – When is surgery recommended for type I gNETs?
Q5 – Can endoscopic treatments be proposed to treat type III gNETs?
Q6 – When should limited or extended surgical treatments be proposed to treat type III gNETs?
Q7 – What scheduled follow-up is recommended after endoscopic/surgical resection of type III gNETs?

Now read the reference material below including where applicable the Functional pancreatic NET syndromes related in some way to Type II gNETs

Reference

Click on the blue link below see read the discussion and the recommendations for each of those questions. 

European Neuroendocrine Tumor Society (ENETS) 2023 guidance paper for gastroduodenal neuroendocrine tumours (NETs) G1–G3

 
First published: 20 May 2023

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