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

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, 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 ellaborate 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, streptozocin, fluoropyrimidines, platinum compounds, etoposide and irinotecan).