ENETS Consensus Guidelines for the Standards of Care in Neuroendocrine Neoplasms: Systemic Therapy-Chemotherapy

Rocio Garcia-Carbonero, Anja Rinke, Juan W. Valle, Nicola Fazio, Martyn Caplin, Vera Gorbounova, Juan O'Connor, Barbro Eriksson, Halfdan Sorbye, Matthew Kulke, Jie Chen, Jenny Falkerby, Frederico Costa, Wouter De Herder, Catherine Lombard-Bohas, Marianne Pavel

Research output: Contribution to journalArticlepeer-review


Systemic chemotherapy is indicated in progressive or bulky advanced pancreatic neuroendocrine tumors (NETs) and in grade 3 (G3) neuroendocrine neoplasms (NENs) as per ENETS guidelines. Chemotherapy may be considered in NETs of other sites (lung, thymus, stomach, colon, and rectum) under certain conditions (e.g., when Ki-67 is at a high level [upper G2 range], in rapidly progressive disease and/or after failure of other therapies, or if somatostatin receptor imaging is negative). An ENETS Consensus Conference was held in Antibes (2015) to elaborate guidelines on the standards of care of different diagnostic procedures and therapeutic interventions in NENs. This article provides guidance on chemotherapy including therapeutic indications, dosing schedules, adverse events (including prevention and management), drug interactions, and evaluation of treatment effect for the chemotherapy agents most commonly used in NENs (streptozocin, dacarbazine, fluoropyrimidines, platinum compounds, etoposide, and irinotecan).

Original languageEnglish
Pages (from-to)281-294
Number of pages14
Issue number3
Publication statusPublished - Sep 1 2017


  • Chemotherapy
  • Dosing schedules
  • Drug interactions
  • Neuroendocrine tumors
  • Standard of care
  • Toxicity

ASJC Scopus subject areas

  • Endocrinology, Diabetes and Metabolism
  • Endocrinology
  • Endocrine and Autonomic Systems
  • Cellular and Molecular Neuroscience


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