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Small Intestine NETs

The latest European Neuroendocrine Tumor Society (ENETS) 2024 guidance paper for the management of well-differentiated small intestine neuroendocrine tumours

As someone who was diagnosed with metastatic small intestine NETs in 2010, I’ve clearly written many words about my own experience and how I was treated.  However, I’ve also researched extensively about these issues including previous guidelines for the management of these common but complex tumours. They are mostly indolent, but they can be aggressive; they are legendar spreaders, often in silence. 

They can cause  damage before and after diagnosis, and it is these tumours that are predomiately involved in mesenteric and retroperitoneal fibrosis, NET related heart disease and so called carcinoid syndrome (despite what you read on many websites, blog sites and onine patient groups).  They can be difficult to diagnose but also incidentially found at all stages of cancer.  However, things are improving, less than 50% of small intestine NETs are now diagnosed at the earlier stages.

I hope you find the latest ENETS thinking of use in your own quest for information on your condition. This guidance was pubished in July 2024. 

Abstract

Both the incidence and prevalence of well-differentiated neuroendocrine tumours from the small intestine (Si-NET) are gradually increasing. Most patients have non-functioning tumours with subtle GI symptoms and tumours are often discovered incidentally by endoscopy or at advanced disease stages by imaging depicting mesenteric lymph node and /or liver metastases while around 30% of the patients present with symptoms of the carcinoid syndrome. Adequate biochemical assessment and staging including functional imaging is crucial for treatment-related decision-making that should take place in an expert multidisciplinary team setting. Preferably, patients should be referred to specialised ENETS Centres of Excellence or centres of high expertise in the field. This guidance paper provides the current evidence and best knowledge for the management of Si-NET grade (G) 1–3 following 10 key questions of practical relevance for the diagnostic and therapeutic decision making.

TABLE 1. Ten (10) clinical questions for managin SI NETs

Q1 – Which features need to be taken into consideration for the management of Si-NET? 

Q2 – Is there any role of circulating biomarker measurement in Si-NET?

Q3 – Which is the most suitable diagnostic and staging work-up for patients with small intestinal NET?

Q4 – What is the role of surgery in localised SI-NET?

Q5 – What is the recommended first-line systemic treatment for advanced disease?

Q6 – What is the recommended treatment beyond first- line SSA therapy?

Q7 – What is the role of locoregional and ablative therapies in advanced Si-NET?

Q8 – Which is the best therapeutic strategy in patients with Carcinoid Syndrome and tumour growth control?

Q9 – What are the most recent developments for Si-NET?

Q10 – What is the recommended follow-up for Si-NET?

Now read the reference material below.  Abstract summary above but great introduction inside the reference. 

Q4 expansion – Mesenteric fibrosis and palliative surgery in small intestinal neuroendocrine tumours

Mesenteric fibrosis (MF) surrounding a mesenteric mass is a hallmark feature of small intestinal neuroendocrine tumours (SI-NETs). Since this can induce intestinal obstruction, oedema and ischaemia, prophylactic resection of the primary tumour and mesenteric mass is often recommended. This study assessed the predictors for mesenteric metastasis and fibrosis and the effect of MF and palliative surgery on survival. A retrospective analysis of 559 patients with pathologically proven SI-NET and available CT-imaging data was performed. Clinical characteristics, presence of mesenteric mass and fibrosis on CT imaging and the effect of palliative abdominal surgery on overall survival were assessed. We found that MF was present in 41.4%. Older age, 5-HIAA excretion ≥67 μmol/24 h, serum CgA ≥121.5 μg/L and a mesenteric mass ≥27.5 mm were independent predictors of MF. In patients ≤52 years, mesenteric mass was less often found in women than in men (39% vs 64%, P = 0.002). Corrected for age, tumour grade, CgA and liver metastasis, MF was not a prognostic factor for overall survival. In patients undergoing palliative surgery, metastasectomy of mesenteric mass or prophylactic surgery did not result in survival benefit. In conclusion, we confirmed known predictors of MF and mesenteric mass and suggest a role for sex hormones as women ≤52 years have less often a mesenteric mass. Furthermore, the presence of MF has no effect on survival in a multivariate analysis, and we found no benefit of metastasectomy of mesenteric mass or prophylactic surgery on overall survival.

Click on the blue link below see read the discussion and the recommendations 

lažević, A., Zandee, W. T., Franssen, G. J. H., Hofland, J., van Velthuysen, M. F., Hofland, L. J., Feelders, R. A., & de Herder, W. W. (2018). Mesenteric fibrosis and palliative surgery in small intestinal neuroendocrine tumours. Endocrine-Related Cancer25(3), 245-254. Retrieved Jun 4, 2025, from https://doi.org/10.1530/ERC-17-0282

Q4 expansion – Vessel-Sparing Lymphadenectomy (VS-LA) Should Be Performed in Small Intestine Neuroendocrine Neoplasms

Primary tumour resection with lymphadenectomy in small intestine neuroendocrine neoplasms potentially requires extensive small bowel resections due to central lymph node metastases and mesenteric fibrosis. Retrograde vessel-sparing lymphadenectomy (VS-LA) might be a sufficient method for avoiding local recurrence and for sparing the small bowel at the same time. We retrospectively analyzed clinical, surgical and pathological data of 50 patients with SI-NENs who exclusively underwent small bowel resections; half of them received conventional lymphadenectomy and 25 underwent VS-LA. VS-LA resulted in shorter resected bowel segments (median 40 cm vs. 65 cm, p = 0.007) with similar rates of local R0 resections (72% vs. 84%) and number of resected lymph nodes (median 13 vs. 13). Postoperative complications occurred significantly less in the vessel-sparing group. VS-LA should be the preferred surgical method in small bowel resections for SI-NENs.

Click on the blue link below see read the discussion and the recommendations.

MDPI and ACS Style

Bartsch, D.K.; Windel, S.; Kanngießer, V.; Jesinghaus, M.; Holzer, K.; Rinke, A.; Maurer, E. Vessel-Sparing Lymphadenectomy Should Be Performed in Small Intestine Neuroendocrine Neoplasms. Cancers 202214, 3610. https://doi.org/10.3390/cancers14153610

More Guidelines

Read guidelines published so far - click picture to browse
click picture to read a blog by Ronny Allan (Stage IV SI NET patient)

Disclaimer

I am not a doctor or any form of medical professional, practitioner or counsellor. None of the information on my website, or linked to my website(s), or conveyed by me on any social media or presentation, should be interpreted as medical advice given or advised by me. 

Neither should any post or comment made by a follower or member of my private group be assumed to be medical advice, even if that person is a healthcare professional. Some content may be generated by AI which can sometimes be misinterpreted.  Please check any references attached.    

Please also note that mention of a clinical service, trial/study or therapy does not constitute an endorsement of that service, trial/study or therapy by Ronny Allan, the information is provided for education and awareness purposes and/or related to Ronny Allan’s own patient experience. This element of the disclaimer includes any complementary medicine, non-prescription over the counter drugs and supplements such as vitamins and minerals.

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