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European Neuroendocrine Tumor Society (ENETS) 2023 guidance paper for digestive neuroendocrine carcinoma |
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| Authors: | Halfdan Sorbye Enrique Grande Marianne Pavel Margot Tesselaar Nicola Fazio Nicholas Simon Reed Ulrich Knigge Emanuel Christ Valentina Ambrosini Anne Couvelard Eva Tiensuu Janson |
| Institution: | 1. Department of Oncology, Haukeland University Hospital and Department of Clinical Science, University of Bergen, Bergen, Norway;2. Department of Medical Oncology, MD Anderson Cancer Center Madrid, Madrid, Spain
Contribution: Data curation, Investigation, Writing - original draft, Writing - review & editing;3. Department of Medicine 1, Friedrich-Alexander University Erlangen-Nürnberg, Erlangen, Germany Contribution: Data curation, Investigation, Writing - original draft, Writing - review & editing;4. Department of Gastrointestinal Oncology, Netherlands Cancer Institute, Amsterdam, The Netherlands Contribution: Data curation, Investigation, Writing - original draft, Writing - review & editing;5. Division of Gastrointestinal Medical Oncology and Neuroendocrine Tumors, European Institute of Oncology, IEO, IRCCS, Milan, Italy Contribution: Writing - original draft, Writing - review & editing;6. Department of Clinical Oncology, Beatson Oncology Center, Glasgow, UK;7. Departments of Surgery and Clinical Endocrinology, ENETS Center of Excellence, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark Contribution: Data curation, Investigation, Writing - original draft, Writing - review & editing;8. Department of Endocrinology, Diabetes and Metabolism, ENETS Center of Excellence, University Hospital of Basel, Basel, Switzerland Contribution: Writing - original draft, Writing - review & editing;9. Nuclear Medicine, Alma Mater Studiorum, University of Bologna, Bologna, Italy IRCCS, Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy Contribution: Writing - original draft, Writing - review & editing;10. Department of Pathology, AP-HP Bichat Hospital, Université Paris Cité, Paris, France Contribution: Data curation, Investigation, Writing - original draft, Writing - review & editing;11. Department of Medical Sciences, Endocrine Oncology, Uppsala University, Uppsala, Sweden Contribution: Conceptualization, Data curation, Investigation, Methodology, Writing - original draft, Writing - review & editing |
| 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. |
| Keywords: | diagnosis digestive NEC neuroendocrine carcinoma neuroendocrine neoplasms |