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

Halfdan Sorbye*, Enrique Grande, Marianne Pavel, Margot Tesselaar, Nicola Fazio, Nicholas Simon Reed, Ulrich Knigge, Emanuel Christ, Valentina Ambrosini, Anne Couvelard, Eva Tiensuu Janson

*Corresponding author for this work


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.

Original languageEnglish
Article numbere13249
JournalJournal of Neuroendocrinology
Issue number3
Pages (from-to)e13249
Publication statusPublished - Mar 2023


  • Carboplatin/therapeutic use
  • Carcinoma, Neuroendocrine/drug therapy
  • Humans
  • Neuroendocrine Tumors/pathology
  • Prognosis
  • Prospective Studies
  • neuroendocrine neoplasms
  • digestive
  • neuroendocrine carcinoma
  • diagnosis
  • NEC


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