1 3 Cancer Chemother Pharmacol DOI 10.1007/s00280-014-2642-2 REVIEW ARTICLE Expert consensus for the management of advanced or metastatic pancreatic neuroendocrine and carcinoid tumors Daniel Castellano · Enrique Grande · Juan Valle · Jaume Capdevila · Diane Reidy‑Lagunes · Juan Manuel O’Connor · Eric Raymond Received: 25 September 2014 / Accepted: 24 November 2014 © Springer-Verlag Berlin Heidelberg 2014 advanced disease may include hepatic artery embolization or ablation, peptide receptor radionuclide therapy and sys- temic chemotherapy. Recent advances regarding the signal- ing pathways involved in tumor development have allowed the development of novel targeted therapies. However, due to the lack of prognostic molecular markers to identify high-risk patients and the absence of a common pathogen- esis in all patients, treatment selection is often empirical. There is therefore a need to establish a consensus for the treatment of this disease and to provide evidence-based clinical recommendations and algorithms to optimize and individualize the treatment and follow-up for these patients. Keywords Carcinoid tumor · Pancreatic neuroendocrine tumor · Radionuclides · Somatostatin analogs · Targeted therapy Introduction Neuroendocrine tumors (NETs) are a heterogeneous fam- ily of tumors with a wide and complex spectrum of clinical behavior [1]. NETs are considered rare tumors, but follow- ing an analysis of surveillance, epidemiology and outcome databases, the estimated annual incidence of carcinoid tumors in 2004 was found to be 5.25 per 100,000 popu- lation, and the 29-year limited duration prevalence in the USA was estimated to exceed 100,000 individuals. These results suggest that NETs are more prevalent than previ- ously reported [2]. Also, recent epidemiological data have revealed that their incidence has increased significantly over the last 30 years, with no significant changes in sur- vival [1, 3]. Neuroendocrine gastrointestinal tumors have classi- cally been divided into endocrine pancreatic tumors and Abstract Neuroendocrine tumors (NETs) are rare tumors that have been increasing in incidence over the last 30 years with no significant changes in survival. As survival of patients with these tumors depends greatly on stage and histology, early diagnosis, classification and stag- ing of tumors in patients in whom NETs are suspected are of great importance. Surgery, either with curative or pal- liative intent, is the mainstay of treatment for localized NETs. Therapeutic options for this disease almost invari- ably include somatostatin analogs to alleviate the symp- toms of excessive hormone secretion. Other approaches for D. Castellano (*) Departamento de Oncología Médica, Hospital Universitario 12 de Octubre, Avda. de Córdoba s/n, 28041 Madrid, Spain e-mail: cdanicas@hotmail.com E. Grande Medical Oncology Department, Ramón y Cajal University Hospital, Madrid, Spain J. Valle Medical Oncology Department, The Christie NHS Foundation Trust, Manchester, UK J. Capdevila Medical Oncology Department, Vall d’Hebron University Hospital, Universitat Autònoma de Barcelona, Barcelona, Spain D. Reidy-Lagunes Gastrointestinal Oncology, Memorial Sloan-Kettering Cancer Center, New York, NY, USA J. M. O’Connor Medical Oncology Department, Fleming Institute, University of Buenos Aires, Buenos Aires, Argentina E. Raymond Medical Oncology Department, Beaujon University Hospital, INSERM U728, Paris 7 University and APHP, Clichy, France