European Neuroendocrine Tumor Society (ENETS) 2023 guidance paper for appendiceal neuroendocrine tumours (aNET)

Appendiceal neuroendocrine tumours (aNET) account for 50%–77% of all appendiceal neoplasms and are mostly identified incidentally following appendicectomy for acute appendicitis or after a laparotomy performed for unrelated reasons. In the great majority, aNET are low grade (>80% grade 1 (G1)) and very rarely, if ever, are associated with a secretory syndrome.

This post is part of my ENETS Guideline series and supplements my Spotlight series article which can be found by clicking here.

TABLE 1. Nine clinical questions for aNET scenarios.

Q1 – What is the current incidence of aNET (G1-3)?

Q2 – Which are the pathological parameters that need to be provided for further decision-making?

Q3 – How common are LN and distant metastases and carcinoid syndrome (CS) in appendiceal NET? Is LN status an acceptable surrogate of OS?

Q4 – Is there any indication for biochemical and/or imaging modalities to be utilised for tumour staging following appendicectomy?

Q5 – Is there any indication for biochemical and/or imaging modalities to be utilised for tumour staging following appendicectomy?

Q6 – When completion oncological surgery is recommended, is RHC always required or could ileocaecal resection be sufficient, and what is the impact of these

Q7 – Is there any need for adjuvant therapy in patients with aNET without distant metastases?

Q8 – How should advanced disease be managed?

Q9 – What is the recommended follow-up protocol after RHC in patients with or without LN+ ? How is follow-up in aNET justified?

Now read the reference material below including where applicable to the 9 questions above.

Reference

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

European Neuroendocrine Tumor Society (ENETS) 2023 guidance paper for appendiceal neuroendocrine tumours (aNET)

Gregory Kaltsas, Thomas Walter, Ulrich Knigge, Christos Toumpanakis, Ana P. Santos, Nehara Begum, Ulrich F. Pape, Marco Volante, Andrea Frilling, Anne Couvelard

First published: 16 August 2023

https://doi.org/10.1111/jne.13332

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