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

    11 Citations (Scopus)
    61 Downloads (Pure)

    Abstract

    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
    Volume172
    DOIs
    Publication statusPublished - Sept-2022

    Keywords

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

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