title: Treatment of symptomatic neuroendocrine tumor syndromes: recent advances and controversies.
abstract: Neuroendocrine tumors(NETs), once thought rare, are increasing in frequency in most countries and receiving increasing-attention. NETs present two-treatment problems. A proportion is aggressive and a proportion has a functional, hormone-excess-state(F-NET), each of which must be treated. Recently, there have been many advances, well-covered in reviews/consensus papers on imaging-NETs; new, novel anti-tumor treatments and understanding their pathogenesis. However, little attention has been paid to advances in the treatment of the hormone-excess-state. These advances are usually reported in case-series, and case-reports with few large studies. In this paper these advances are reviewed. Areas covered: Advances in the last 5-years are concentrated on, but a review of literature from the last 10-years was performed. PubMed and other databases (Cochrane, etc.) were searched for F-NET-syndromes including carcinoid-syndrome, as well as meeting-abstracts on NETs. All advances that controlled hormone-excess-states or facilitated-control were covered. These include new medical-therapies [serotonin-synthesis inhibitors(telotristat), Pasireotide, new agents for treating ACTHomas], increased dosing with conventional therapies (octreotide-LAR, Lanreotide-Autogel), mTor inhibitors(everolimus), Tyrosine-kinase inhibitors(sunitinib),cytoreductive surgery, liver-directed therapies (embolization, chemoembolization, radioembolization, RFA), peptide radio-receptor-therapy(PRRT) and131I-MIBG, ablation of primary F-NETs. Expert opinion: Although many of the newer therapies controlling the hormone-excess-states in F-NETs are reported in relatively few patients, all the approaches show promise. Their description also generates some controversies/unresolved areas,such as the order of these new treatments, their longterm-efficacy, and effectiveness of combinations which may require large,controlled studies.
authors.affiliation: a Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences , Kyushu University , Fukuoka , Japan.
authors.forename: Tetsuhide
authors.initials: T
authors.lastname: Ito
authors.affiliation: a Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences , Kyushu University , Fukuoka , Japan.
authors.forename: Lingaku
authors.initials: L
authors.lastname: Lee
authors.affiliation: b Digestive Diseases Branch , NIDDK, NIH , Bethesda , MD , USA.
authors.forename: Robert T
authors.initials: RT
authors.lastname: Jensen
mesh_terms: D000818:Animals; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage*; D000068338:Everolimus / Q000627:therapeutic use; D006801:Humans; D007211:Indoles / Q000627:therapeutic use; D058990:Molecular Targeted Therapy / Q000379:methods / Q000639:trends; D018358:Neuroendocrine Tumors / Q000175:diagnosis / Q000188:drug therapy*; D015282:Octreotide / Q000627:therapeutic use; D011758:Pyrroles / Q000627:therapeutic use; D013004:Somatostatin / Q000008:administration & dosage* / Q000031:analogs & derivatives* / Q000627:therapeutic use; D000077210:Sunitinib; D013577:Syndrome; D016896:Treatment Outcome
chemical_list: D007211:Indoles; D011758:Pyrroles; D013004:Somatostatin; C517782:pasireotide; D000068338:Everolimus; D015282:Octreotide; D000077210:Sunitinib
keywords: Acid hypersecretion; Lanreotide; SIRT; Zollinger-Ellison syndrome; carcinoid syndrome; chemoembolization; chemotherapy; cytoreductive surgery; embolization; gastrinoma; insulinoma; multiple endocrine Neoplasia type 1; neuroendocrine tumor; octreotide; pancreatic endocrine tumor; peptide radio-receptor therapy; radio-frequency ablation; radioembolization; somatostatin analogues; telotristat