European Neuroendocrine Tumor Society (ENETS) 2023 guidance paper for digestive neuroendocrine carcinoma

Abstract

This ENETS guidance paper, developed by a multidisciplinary working group, provides up‐to‐date and practical advice on the diagnosis and management of digestive neuroendocrine carcinoma, based on recent developments and study results. These recommendations aim to pave the road for more standardized care for our patients resulting in improved outcomes. Prognosis is generally poor for digestive NEC, most are advanced at diagnosis and median survival in metastatic disease is 11–12 months. Surgery can be of benefit for localized disease after extensive preoperative imaging. Carboplatin in combination with etoposide is recommended as first‐line treatment for metastatic disease. Irinotecan with fluoropyrimidines has the best evidence as second‐line treatment. Immunotherapy plays a minor role in biomarker‐unselected patients. Molecular profiling if available is encouraged to identify new targets. More prospective clinical trials are highly needed to fulfil the unmet needs in this field, especially on new predictive and prognostic biomarkers and to improve survival of patients with advanced disease.

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

“…The indication and the timing of surgery in MEN1-ZES are controversial, since it has been shown that patients with no imageable pancreatic tumour, who did not undergo surgery, have a good prognosis with 5-year survival rates around 90% and a 10 year survival rate of 54% in cases of disseminated distant metastases 72 [3]. One has to keep in mind that patients with MEN1-ZES always have small duodenal gastrinomas and almost never pancreatic gastrinomas, as demonstrated by pathological studies 60 [2, A].…”

“…The reader is referred to the ENETS 2023 guidance papers on non-functioning (NF-)Pan-NET and gastroduodenal NET for primary oncological treatment of pancreatic or duodenal NET, whereas management of poorly differentiated counterparts is discussed in the ENETS 2023 guidance paper for digestive NEC. 3 All recommendations in this guidance paper are graded according to the level of evidence (1)(2)(3)(4)(5) and strength of recommendation (A-D) (Table 2).…”

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

“…For details please see text recommendation 2.8.1. 5 In cases with more than one risk factor even with normal imaging a careful discussion with the patient is necessary to decide on follow-up or oncological resection; risk factors are: size >1 cm, G > 1; L1; V1. eFTR, endoscopic full thickness resection; ESD, endoscopic submucosal dissection; FU, follow-up; mEMR, modified endoscopic mucosal resection (for example cap EMR); TAMIS, transanal minimallly invasive surgery.…”

“…Anal canal neuroendocrine neoplasm (NEN) are usually NEC and often MiNEN with a squamous carcinoma component 4 . NEC and MINEN are discussed in a separate guidance paper 5 …”

“…4 NEC and MINEN are discussed in a separate guidance paper. 5 1.1 | Pathology NEN of the large intestine are classified and staged according to the current WHO classification of digestive tumours, 2022 update and 2017 AJCC Cancer Staging system. 3,6,7 Below are summarised the major features (Tables 1 and 2) and the link to the ENETS proposal for synoptic reporting: 8 https://www.enets.org/standardised-reports.html?file=files/enets/ customer/media/Standardised%20reports/Standard%20Report% 20Pathology_ColoRectal%20NEN_V4.pdf&cid=2784.…”