European Neuroendocrine Tumour Society (ENETS) 2023 guidance paper for nonfunctioning pancreatic neuroendocrine tumours

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There is very much a multi-disciplinary team (MDT) approach to treating Neuroendocrine Tumours (NET) in Europe and many other places.  In Europe and the extra-Europe CoEs in Australia, Israel and the USA, this is centred on the establishment of the Centre of Excellence programme that has been running since 2009.  But underneath that are some excellent MDTs not yet accredited who may still utilise ENETs guidelines to treat and manage their patients. 

This 2023 guideline paper sets the scene for an important and complex group of tumours – pancreatic NETs.  It’s well known and accepted that the majority of pancreatic NETs are non-functional, depending on where you look, anything between 50 and 85%. “Non-functional” means that the tumour doesn’t produce a sufficient set of symptoms to cause a hormonal syndrome.

While the diagnosis of functionality relies on the presence of a hormonal syndrome, the NET-diagnosis continues to rely on histological or cytological analysis.  There is a separate guidance paper on functional pancreatic NETs – click here to read that. 

Prior to 2022/23, the most recent and comprehensive set of guidelines were based on the 2016 series which were behind a subscription wall. I have had access to those via my ENETS membership and quoted them many times in my patient group and citing them in my spotlight on NENs series.  I am very pleased to see many of the 2022/23 guidance papers made public and am happy to share with you via my blog.  

This ENETS guidance paper for well-differentiated nonfunctioning pancreatic neuroendocrine tumours (NF-Pan-NET) has been developed by a multidisciplinary working group and provides up-to-date and practical advice on the management of these tumours.

Using the extensive experience of centres treating patients with NF-Pan-NEN, the authors of this guidance paper discuss 10 troublesome questions in everyday clinical practice. Our many years of experience in this field are still being verified in the light of the results of new clinical, which set new ways of proceeding in NEN. The treatment of NF-Pan-NEN still requires a decision of a multidisciplinary team of specialists in the field of neuroendocrine neoplasms.  

I see from watching exchanges between ENET specialists and other regions (North America etc), I see there is a wide consensus to be found in each other’s guidelines and recommendations.  I also note they both attend each other’s annual conferences, listening, contributing and presenting. This has to be a good thing for patients. Nonetheless, those not being treated in ENETS Centres of Excellence or associated MDTs, should refer to their own regional guidelines. 

See Table 1 below on the management of these neoplasms

TABLE 1. Ten major questions on management of nonfunctioning pancreatic neuroendocrine tumours.

  1. How should we define and characterise a NF-Pan-NET patient at clinical presentation?
  2. Which biochemical tests should be performed in a patient with NF-Pan-NET?
  3. Which is the most suitable imaging work-up for NF-Pan-NET patients?
  4. What is the appropriate surgical management of NF-Pan-NET?
  5. What is the role of PRRT in patients with NF-Pan-NET?
  6. What is the role of biotherapy and molecular targeted therapies in patients with advanced NF-Pan-NET?
  7. What is the role of chemotherapy in patients with advanced NF-Pan-NET?
  8. In the setting of advanced disease, which is the most suitable first-line systemic therapy? Which sequence of treatments should be used?
  9. Is there a specific work-up in MEN1-associated NF-Pan-NET patients?
  10. What is the recommended follow-up in NF-Pan-NET patients?

Now read the reference material below


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

European Neuroendocrine Tumour Society (ENETS) 2023 guidance paper for nonfunctioning pancreatic neuroendocrine tumours

Beata Kos-KudłaJusto P. CastañoTimm DeneckeEnrique GrandeAndreas KjaerAnna KoumarianouLouis de MestierStefano PartelliAurel PerrenStefan StättnerJuan W. ValleNicola Fazio

First published: 05 October 2023

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