Louis de Mestier1, Come Lepage2, Eric Baudin3, Romain Coriat4, Frédéric Courbon5, Anne Couvelard6, Christine Do Cao7, Eric Frampas8, Sébastien Gaujoux9, Rodica Gincul10, Pierre Goudet11, Catherine Lombard-Bohas12, Gilles Poncet13, Denis Smith14, Philippe Ruszniewski1, Thierry Lecomte15, Olivier Bouché16, Thomas Walter12, Guillaume Cadiot17. 1. Department of Gastroenterology-Pancreatology, ENETS Centre of Excellence, Beaujon Hospital (APHP) and Université de Paris, Clichy, France. 2. Department of Gastroenterology and Digestive Oncology, Dijon University Hospital, EPICAD INSERM LNC UMR 1231, University of Burgundy Dijon, France. 3. Department of Nuclear Medicine and Endocrine Oncology, Gustave-Roussy Institute, Villejuif, France. 4. Department of Gastroenterology and Digestive Oncology, Cochin Hospital (APHP) and Université de Paris, Paris, France. 5. Department of Nuclear Medicine, Institut Universitaire du Cancer de Toulouse-Oncopole, Toulouse, France. 6. Department of Pathology of Bichat-Beaujon Hospitals (APHP), ENETS Centre of Excellence and Université de Paris, Paris, France. 7. Department of Endocrinology, Hôpital Claude Huriez, Lille University Hospital, Lille, France. 8. Department of Radiology, Nantes University Hospital, Nantes, France. 9. Department of Endocrine and Pancreatic Surgery, Cochin Hospital (APHP) and University of Paris, Paris, France. 10. Department of Gastroenterology, Ramsay Générale de Santé, Hôpital Privé Jean Mermoz, Lyon, France. 11. Department of Endocrine Surgery, Dijon University Hospital and University of Burgundy, Dijon, France. 12. Department of Oncology, ENETS Centre of Excellence, Hospices Civils de Lyon and Lyon University, Lyon, France. 13. Department of Digestive Surgery, ENETS Centre of Excellence, Edouard Herriot Hospital and Lyon University, Lyon, France. 14. Department of Digestive Oncology, Haut-Lévèque Hospital and University of Bordeaux, Pessac, France. 15. Department of Gastroenterology and Digestive Oncology, Tours University Hospital, Tours, France. 16. Department of Hepato-Gastroenterology and Digestive Oncology, Robert Debré University Hospital, Reims, France. 17. Department of Hepato-Gastroenterology and Digestive Oncology, Robert Debré University Hospital, Reims, France. Electronic address: gcadiot@chu-reims.fr.
Abstract
INTRODUCTION: This document is a summary of the French Intergroup guidelines regarding the management of digestive neuroendocrine neoplasms (NEN) published in February 2020 (www.tncd.org). METHODS: All French medical societies involved in the management of NEN took part in this work. Recommendations were graded into four categories (A, B, C or D), according to the level of evidence found in the literature until May 2019. RESULTS: The management of NEN is challenging because of their heterogeneity and the increasing complexity of diagnostic and therapeutic procedures. Pathological analysis is required for their diagnostic and prognostic characterization, which mainly relies on differentiation, grade and stage. The two main emergency situations are functioning syndromes and poorly-differentiated carcinoma. Chromogranin A is the main biochemical marker of NET, although of limited clinical interest. Initial characterization relies on morphological and isotopic imaging. The treatment of localized NET relies on watchful follow-up and local or surgical resection depending on its supposed aggressiveness. Treatment options for metastatic disease include surgery, somatostatin analogues, chemotherapy, targeted therapies, organ-driven locoregional therapies and peptide-receptor radionuclide therapy. As specific predictive factors of treatment efficacy are yet to be identified and head-to-head comparisons have not or only rarely been performed, the therapeutic strategy currently depends on prognostic factors. Cumulative toxicity and the impact of treatment on quality of life must be considered since survival is relatively long in most patients with NET. CONCLUSION: These guidelines are proposed to achieve the most beneficial therapeutic strategy in clinical practice as the therapeutic landscape of NEN is becoming ever more complex. These recommendations are permanently being reviewed.
INTRODUCTION: This document is a summary of the French Intergroup guidelines regarding the management of digestive neuroendocrine neoplasms (NEN) published in February 2020 (www.tncd.org). METHODS: All French medical societies involved in the management of NEN took part in this work. Recommendations were graded into four categories (A, B, C or D), according to the level of evidence found in the literature until May 2019. RESULTS: The management of NEN is challenging because of their heterogeneity and the increasing complexity of diagnostic and therapeutic procedures. Pathological analysis is required for their diagnostic and prognostic characterization, which mainly relies on differentiation, grade and stage. The two main emergency situations are functioning syndromes and poorly-differentiated carcinoma. Chromogranin A is the main biochemical marker of NET, although of limited clinical interest. Initial characterization relies on morphological and isotopic imaging. The treatment of localized NET relies on watchful follow-up and local or surgical resection depending on its supposed aggressiveness. Treatment options for metastatic disease include surgery, somatostatin analogues, chemotherapy, targeted therapies, organ-driven locoregional therapies and peptide-receptor radionuclide therapy. As specific predictive factors of treatment efficacy are yet to be identified and head-to-head comparisons have not or only rarely been performed, the therapeutic strategy currently depends on prognostic factors. Cumulative toxicity and the impact of treatment on quality of life must be considered since survival is relatively long in most patients with NET. CONCLUSION: These guidelines are proposed to achieve the most beneficial therapeutic strategy in clinical practice as the therapeutic landscape of NEN is becoming ever more complex. These recommendations are permanently being reviewed.
Authors: Maxime Lelièvre; Elizabeth Katherine Anna Triumbari; Hedia Brixi; Marine Perrier; Guillaume Cadiot; Sophie Deguelte; David Morland Journal: Endocrine Date: 2022-10-07 Impact factor: 3.925