顶Check for updatesReceived:13 February 2023 Revised:20 February 2023Accepted:20 February 2023D0:10.1111/jne.13249CLINICALGUIDELINEJoumal of NeuroendocrinologyWILEYEuropean Neuroendocrine Tumor Society (ENETS)2023guidance paper for digestive neuroendocrine carcinomaHalfdan Sorbye1 Enrique Grande2 Marianne Pavel3 Margot Tesselaar4Nicola Fazio5 Nicholas Simon Reeds Ulrich Knigge7 Emanuel Christ3Valentina Ambrosini10 Anne Couvelard 11 Eva Tiensuu Janson12Department of Oncology.Haukeland University Hospital and Department of Cinical Science.University of Bergen.Bergen.Norway2Department of Medical Oncology.MD Anderson Cancer Center Madrid,Madrid.Spain3Department of Medicine 1,Friedrich-Alexander University Erlangen-Numberg.Erlangen,GermanyDepartment of Gastrointestinal Oncology.Netherlands Cancer Institute,Amsterdam,The NetherlandsSDivision of Gastrointestinal Medical Oncology and Neuroendocrine Tumors.European Institute of Oncology.EO.IRCCS.Milan.ItalyDepartment of Clinical Oncology.Beatson Oncology Center.Glasgow,UKDepartments of Surgery and Cinical Endocrinology.ENETS Center of Excellence.Copenhagen University Hospital,Rigshospitalet,Copenhagen.DenmarkDepartment of Endocrinology.Diabetes and Metabolism,ENETS Center of Excellence.University Hospital of Basel,Basel SwitzerlandNuclear Medicine,Alma Mater Studiorum,University of Bologna,Bologna,ItalyRCCS,Azienda Ospedaliero-Universitaria di Bologna,Bologna ItalyDepartment of Pathology.AP-HP Bichat Hospital Universite Paris Cite,Paris,France12Department of Medical Sciences,Endocrine Oncology.Uppsala University.Uppsala,SwedenCorrespondenceHalfdan Sorbye,Department of Oncology,AbstractHaukeland University Hospital,5021 BergenNorway.This ENETS guidance paper,developed by a multidisciplinary working group,providesEmail:halfdan.sorbye@helse-bergen.noup-to-date and practical advice on the diagnosis and management of digestive neuro-endocrine carcinoma,based on recent developments and study results.These recom-mendations aim to pave the road for more standardized care for our patientsresulting in improved outcomes.Prognosis is generally poor for digestive NEC,mostare advanced at diagnosis and median survival in metastatic disease is 11-12months.Surgery can be of benefit for localized disease after extensive preoperativeimaging.Carboplatin in combination with etoposide is recommended as first-linetreatment for metastatic disease.Irinotecan with fluoropyrimidines has the bestevidence as second-line treatment.Immunotherapy plays a minor role in biomarker-unselected patients.Molecular profiling if available is encouraged to identify newtargets.More prospective clinical trials are highly needed to fulfil the unmet needsin this field,especially on new predictive and prognostic biomarkers and to improvesurvival of patients with advanced disease.KEYWORDSdiagnosis,digestive,NEC,neuroendocrine carcinoma,neuroendocrine neoplasmsThis is an open access artide under the terms of theCreativeCommons Attribution License,which p
