European Neuroendocrine Tumor Society 2023 guidance paper for functioning pancreatic neuroendocrine tumour syndromes

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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 minority of pancreatic NETs are non-functional, depending on where you look, anything between 50 and 85%. This means functional pancreatic NETs are less common than non-functional. The reason for emphasis is that patient support groups may misrepresent the real figures as those joining tend to be looking for more support.  I also emphasise the existing of several pancreatic NET syndromes to water down the claim that many pancreatic NETs have carcinoid syndrome when the numbers are really quite low.

So, what does functional mean? 

“Functional” means that the tumour doesn’t make sufficient hormones to produce a set of symptoms (a syndrome).

While the diagnosis of functionality relies on the presence of a hormonal syndrome, the NET diagnosis relies on histological or cytological analysis.  There is a separate guidance paper on non-functional pancreatic NETs (click here).

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 functioning pancreatic neuroendocrine tumours (F-Pan-NET) has been developed by a multidisciplinary working group and provides up-to-date and practical advice on the management of these tumours and syndromes.

Using the extensive experience of centres treating patients with functional Pan-NEN, the authors of this guidance paper discuss 9 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 F-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. Nine clinical questions for functional Pan-NET scenarios.

1. How should we define and characterise an insulinoma and differentiate this from other causes of hypoglycaemia?

2. Which biochemical tests should be performed in patients with clinical suspicion of gastrinoma?

3. Which biochemical test should be performed to diagnose rare functioning syndromes (glucagonoma, VIPoma, ACTHoma, carcinoid syndrome, PTHrPoma, calcitoninoma, GHRHoma, somatostatinoma) in patients?

4. Which patients with functioning Pan-NET syndromes should be referred for genetic counselling?

5. What is the difference in the imaging work-up of functioning Pan-NET as compared to non-functioning Pan-NET?

6. What is the appropriate surgical management of localised insulinoma?

7. What is the appropriate surgical management of localised gastrinoma?

8. What is the appropriate management of patients with advanced disease and functioning Pan-NET syndromes?

9. What is the recommended follow-up in functioning Pan-NET syndromes after curative resection?

Now read the reference material below

Reference

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

European Neuroendocrine Tumor Society 2023 guidance paper for functioning pancreatic neuroendocrine tumour syndromes

Johannes Hofland ¹, Massimo Falconi ², Emanuel Christ ^3 4, Justo P Castaño ^5 6 7 8, Antongiulio Faggiano ⁹, Angela Lamarca ^10 11, Aurel Perren ¹², Simona Petrucci ^13 14, Vikas Prasad ¹⁵, Philippe Ruszniewski ¹⁶, Christina Thirlwell ¹⁷, Marie-Pierre Vullierme ¹⁸, Staffan Welin ¹⁹, Detlef K Bartsch ²⁰

DOI: 10.1111/jne.13318

More Guidelines

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Thanks for reading.

Ronny

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