Long-term survival in patients with gastroenteropancreatic neuroendocrine neoplasms: A population-based study

Iris N Poleé, Bregtje C M Hermans, Jan Maarten van der Zwan, Stefan A W Bouwense, Marcus W Dercksen, Ferry A L M Eskens, Bastiaan Havekes, Johannes Hofland, Thomas M A Kerkhofs, Heinz-Josef Klümpen, Loes M Latten-Jansen, Ernst-Jan M Speel, Frederik A Verburg, Annemiek M E Walenkamp, Sandra M E Geurts, Judith de Vos-Geelen*

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review


BACKGROUND: Gastroenteropancreatic (GEP) neuroendocrine neoplasms (NENs) comprise a group of rare malignant tumours with heterogeneous behaviour. This study aimed to assess long-term survival and prognostic factors associated with survival, in order to optimise counselling.

PATIENTS AND METHODS: This population-based study included all GEP-NENs diagnosed between 1989 and 2016 in the Netherlands, selected from the Netherlands Cancer Registry. Overall survival (OS) and relative survival (RS) were calculated. A Cox Proportional Hazard analysis was used to identify prognostic factors (gender, age, tumour stage, location and treatment) for OS. Analyses were stratified by metastatic disease status and tumour grade.

RESULTS: In total, 9697 patients were included. In grade 1, 2 and 3 non-metastatic GEP-NENs (N = 6544), 5-year OS and RS were 81% and 88%, 78% and 83%, and 26% and 30%, respectively. In grade 1 non-metastatic GEP-NENs 10-year OS and RS were 68% and 83%. In grade 1, 2 and 3 metastatic GEP-NENs (N = 3153), 5-year OS and RS rates were 47% and 52%, 38% and 41%, and 5% and 5%, respectively. The highest (relative) survival rates were found in appendicular and rectal NENs, demonstrating 10-year OS and RS of 87% and 93%, and 81% and 95%, respectively.

CONCLUSIONS: These long-term follow-up data demonstrate significant differences in survival for different grades, tumour stage, and primary origin of GEP-NENs, with the most favourable overall and RS rates in patients with non-metastatic grade 1 appendicular and rectal NENs. This study demonstrates unique long-term OS and RS rates using combined stratification by tumour site, grade and stage.

Original languageEnglish
Pages (from-to)252-263
Number of pages12
JournalEuropean Journal of Cancer
Early online date5 Jul 2022
Publication statusPublished - Sept 2022


  • Carcinoid
  • Gastrointestinal
  • Neuroendocrine tumours
  • Pancreas
  • Population-based
  • Prognosis
  • Survival

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