title: Evolution in the treatment of gastroenteropancreatic-neuroendocrine neoplasms, focus on systemic therapeutic options: a systematic review.
abstract: Neuroendocrine neoplasms (NENs) are a group of heterogeneous tumors. The present review discusses current therapeutic strategies for the treatment of gastro-entero-pancreatic NEN. Several systemic options are currently available, including medical systemic chemotherapy, biological drugs, somatostatin analogs and peptide receptor radionuclide therapy. The carcinoid syndrome can be adequately controlled with somatostatin analogs; chemotherapy has shown positive outcomes in poor prognosis patients, and peptide receptor radionuclide therapy is a promising treatment based on the use of radioisotopes for advanced disease expressing somatostatin receptors. Targeted therapies, such as multikinase inhibitors and monoclonal antibodies are also recommended or under evaluation for the treatment of advanced NENs, but some critical issues in clinical practice remain unresolved. Depending upon the development of the disease, a multimodal approach is recommended. The treatment strategy for metastatic patients should be planned by a multidisciplinary team in order to define the optimal sequence of treatments.
authors.affiliation: Department of Medical Oncology, ENETS Center of Excellence, Fondazione IRCCS 'Istituto Nazionale dei Tumori', Milan, Italy.
authors.forename: Sara
authors.initials: S
authors.lastname: Pusceddu
authors.affiliation: Department of Medical Oncology, ENETS Center of Excellence, Fondazione IRCCS 'Istituto Nazionale dei Tumori', Milan, Italy.
authors.forename: Filippo
authors.initials: F
authors.lastname: De Braud
authors.affiliation: Department of Pharmacy, ENETS Center of Excellence, Fondazione IRCCS 'Istituto Nazionale dei Tumori', Milan, Italy.
authors.forename: Fabrizio
authors.initials: F
authors.lastname: Festinese
authors.affiliation: Department of Medical Oncology, ENETS Center of Excellence, Fondazione IRCCS 'Istituto Nazionale dei Tumori', Milan, Italy.
authors.forename: Cristina
authors.initials: C
authors.lastname: Bregant
authors.affiliation: Department of Nuclear Medicine, ENETS Center of Excellence, Fondazione IRCCS 'Istituto Nazionale dei Tumori', Milan, Italy.
authors.forename: Alice
authors.initials: A
authors.lastname: Lorenzoni
authors.affiliation: Department of Nuclear Medicine, ENETS Center of Excellence, Fondazione IRCCS 'Istituto Nazionale dei Tumori', Milan, Italy.
authors.forename: Marco
authors.initials: M
authors.lastname: Maccauro
authors.affiliation: Department of Pathology, ENETS Center of Excellence, Fondazione IRCCS 'Istituto Nazionale dei Tumori', Milan, Italy.
authors.forename: Massimo
authors.initials: M
authors.lastname: Milione
authors.affiliation: Department of Medical Oncology, ENETS Center of Excellence, Fondazione IRCCS 'Istituto Nazionale dei Tumori', Milan, Italy.
authors.forename: Laura
authors.initials: L
authors.lastname: Concas
authors.affiliation: Department of Medical Oncology, ENETS Center of Excellence, Fondazione IRCCS 'Istituto Nazionale dei Tumori', Milan, Italy.
authors.forename: Barbara
authors.initials: B
authors.lastname: Formisano
authors.affiliation: Department of Medical Oncology, ENETS Center of Excellence, Fondazione IRCCS 'Istituto Nazionale dei Tumori', Milan, Italy.
authors.forename: Livia
authors.initials: L
authors.lastname: Leuzzi
authors.affiliation: Department of Surgery & liver transplantation, ENETS Center of Excellence, Fondazione IRCCS 'Istituto Nazionale dei Tumori', Milan, Italy.
authors.forename: Vincenzo
authors.initials: V
authors.lastname: Mazzaferro
authors.affiliation: Department of Medical Oncology, ENETS Center of Excellence, Fondazione IRCCS 'Istituto Nazionale dei Tumori', Milan, Italy.
authors.forename: Roberto
authors.initials: R
authors.lastname: Buzzoni
mesh_terms: D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D015972:Gene Expression Regulation, Neoplastic; D006801:Humans; D058990:Molecular Targeted Therapy; D018358:Neuroendocrine Tumors / Q000188:drug therapy* / Q000235:genetics / Q000473:pathology; D010190:Pancreatic Neoplasms / Q000188:drug therapy* / Q000235:genetics / Q000473:pathology; D011868:Radioisotopes / Q000627:therapeutic use; D018000:Receptors, Peptide / Q000627:therapeutic use*; D017481:Receptors, Somatostatin / Q000096:biosynthesis / Q000235:genetics; D013004:Somatostatin / Q000031:analogs & derivatives / Q000627:therapeutic use*
chemical_list: D000911:Antibodies, Monoclonal; D011868:Radioisotopes; D018000:Receptors, Peptide; D017481:Receptors, Somatostatin; D013004:Somatostatin
keywords: PRRT; chemotherapy; neuroendocrine tumor; somatostatin analogs; target therapy