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Pancreatic Neoplasms: HELP
Articles by Guillaume Cadiot
Based on 20 articles published since 2010
(Why 20 articles?)
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Between 2010 and 2020, G. Cadiot wrote the following 20 articles about Pancreatic Neoplasms.
 
+ Citations + Abstracts
1 Guideline Malignant insulinoma: recommendations for characterisation and treatment. 2013

Baudin, Eric / Caron, Philippe / Lombard-Bohas, Catherine / Tabarin, Antoine / Mitry, Emmanuel / Reznick, Yves / Taieb, David / Pattou, François / Goudet, Pierre / Vezzosi, Delphine / Scoazec, Jean-Yves / Cadiot, Guillaume / Borson-Chazot, Françoise / Do Cao, Christine / Anonymous2980768 / Anonymous2990768. ·Service de médecine nucléaire et d'oncologie endocrinienne, institut Gustave-Roussy, 94800 Villejuif, France. ·Ann Endocrinol (Paris) · Pubmed #23993836.

ABSTRACT: -- No abstract --

2 Clinical Trial Evaluating bevacizumab in combination with FOLFIRI after the failure of platinum-etoposide regimen in patients with advanced poorly differentiated neuroendocrine carcinoma: The PRODIGE 41-BEVANEC randomized phase II study. 2018

Walter, Thomas / Malka, David / Hentic, Olivia / Lombard-Bohas, Catherine / Le Malicot, Karine / Smith, Denis / Ferru, Aurélie / Assenat, Eric / Cadiot, Guillaume / Lievre, Astrid / Kurtz, Jean-Emmanuel / Dahan, Laetitia / Dubreuil, Olivier / Hautefeuille, Vincent / Lepere, Céline / Gangloff, Alice / Elhajbi, Farid / Coriat, Romain / Roquin, Guillaume / Bouarioua, Nadia / Granger, Victoire / Scoazec, Jean-Yves / Lepage, Côme. ·Department of Medical Oncology, Edouard Herriot Hospital, Hospices Civils de Lyon, Lyon, France. Electronic address: thomas.walter@chu-lyon.fr. · Gastrointestinal Oncology Department, Gustave Roussy Institute, Villejuif, France. · Gastroenterology-Pancreatology Department, Beaujon Hospital, PMAD, Clichy, France. · Department of Medical Oncology, Edouard Herriot Hospital, Hospices Civils de Lyon, Lyon, France. · Fédération Francophone de Cancérologie Digestive, Dijon, France. · Hepatogastroenterology and Digestive Oncology Department, Haut-Lévèque, University Hospital of Bordeaux, Pessac, France. · Pôle régional de cancérologie, University Hospital of Poitiers, Poitiers, France. · Medical Oncology Department, University Hospital St Eloi, Montpellier, France. · Department of Hepatogastroenterology and Digestive Oncology, Robert Debré Hospital, University Hospital of Reims, Reims, France. · Service des maladies de l'appareil digestif, University Hospital of Pontchaillou, Rennes, France. · Oncology Department, Nouvel Hospital Civil, University Hospital of Strasbourg, Strasbourg, France. · Digestive Oncology Department, University Hospital Timone, Marseille, France. · Hepatogastroenterology and Digestive Oncology Department, Pitié Salpêtrière Hospital, Paris, France. · Gastroenterology and Digestive Oncology, Amiens University Hospital, Amiens, France. · European Georges Pompidou Hospital, Paris, France. · Gastroenterology Department, University Hospital of Rouen, Rouen, France. · Oncology Department, Oscar Lambret Center, Lille, France. · Gastroenterology Department, Cochin Hospital, Paris, France. · Gastroenterology & Digestive Oncology, University Hospital of Angers, Angers, France. · Service de gastroentérologie et oncologie digestive, hôpital Nord, Saint Priest en Jarez, France. · Hepatogastroenterology Department, Michallon Hospital, University Hospital of Grenoble, Grenoble, France. · Gustave Roussy Cancer Campus, Department of Surgical and Molecular Pathology, Villejuif Cedex, France; Université Paris Saclay, Université Paris Sud XI, Faculté de Médecine de Bicêtre, Le Kremlin-Bicêtre, France. · Gastrointestinal Oncology Department, Gustave Roussy Institute, Villejuif, France; Gastroenterology & Digestive Oncology, University Hospital Le Bocage, Dijon, France. ·Dig Liver Dis · Pubmed #29258812.

ABSTRACT: INTRODUCTION: Patients with gastroenteropancreatic (GEP), metastatic or locally advanced, non-resectable, grade 3 poorly-differentiated neuroendocrine carcinoma (NEC) are treated with cisplatin (or carboplatin)-etoposide in first-line palliative chemotherapy (CT1). However, nearly all patients will develop resistance and there is no standard second-line treatment. AIM: PRODIGE 41-BEVANEC is an academic randomized, phase II study designed to evaluate the efficacy of bevacizumab in combination with FOLFIRI after failure of CT1 in unknown primary NEC and GEP-NEC. MATERIALS AND METHODS: The main eligibility criteria are age ≥18 years, metastatic (synchronous or metachronous) or locally advanced, non-resectable, grade 3 GEP-NEC, and documented progressive disease during or after CT1 therapy. RESULTS: A total of 124 patients will be randomly assigned (1:1) to receive either 5 mg/kg bevacizumab with FOLFIRI, or FOLFIRI alone, every 14 days until disease progression or unacceptable toxicity. The hypothesis is to demonstrate a 6-month overall survival for at least 50% of the patients in bevacizumab arm versus 35% in the control arm (FOLFIRI alone). Secondary endpoints are objective response, response duration, progression-free survival, toxicity, and biochemical response. CONCLUSION: The study is currently opened in France (NCT02820857). The first patient was randomized on September 6, 2017.

3 Clinical Trial Long-term Follow-up of MEN1 Patients Who Do Not Have Initial Surgery for Small ≤2 cm Nonfunctioning Pancreatic Neuroendocrine Tumors, an AFCE and GTE Study: Association Francophone de Chirurgie Endocrinienne & Groupe d'Etude des Tumeurs Endocrines. 2018

Triponez, Frederic / Sadowski, Samira M / Pattou, François / Cardot-Bauters, Catherine / Mirallié, Eric / Le Bras, Maëlle / Sebag, Frédéric / Niccoli, Patricia / Deguelte, Sophie / Cadiot, Guillaume / Poncet, Gilles / Lifante, Jean-Christophe / Borson-Chazot, Françoise / Chaffanjon, Philippe / Chabre, Olivier / Menegaux, Fabrice / Baudin, Eric / Ruszniewski, Philippe / Du Boullay, Hélène / Goudet, Pierre. ·Department of Thoracic and Endocrine Surgery, University Hospitals of Geneva and Faculty of Medicine of Geneva, Geneva, Switzerland. · Department of General and Endocrine Surgery, University Hospital of Lille, Lille, France. · Department of Endocrinology, University Hospital of Lille, Lille, France. · Department of Digestive and Endocrine Surgery, University Hospital of Nantes, Nantes, France. · Department of Endocrinology, University Hospital of Nantes, Nantes, France. · Department of Endocrine Surgery, La Conception University Hospital, Marseille, France. · Institut Paoli-Calmettes, Marseille, France. · Department of Digestive and Endocrine Surgery, University Hospital of Reims, Reims, France. · Department of Hepato-Gastroenterology and Digestive Oncology, University Hospital of Reims, Reims, France. · Department of Surgery, University Hospital of Lyon, Lyon, France. · Department of Digestive and Endocrine Surgery, University Hospital of Lyon Sud and 2/EA 7425 HESPER, Health Services and Performance Research, University Claude Bernard Lyon 1, Lyon, France. · Department of Endocrinology, Hospital Louis Pradel, University Lyon I, Lyon, France. · Department of Thoracic, Vascular and Endocrine Surgery, University Hospital of Grenoble, Grenoble, France. · Department of Endocrinology and Diabetology, University Hospital of Grenoble, Grenoble, France. · Department of Digestive and Endocrine Surgery, University Hospital La Pitié Salpétrière, Paris, France. · Department of Nuclear Medicine and Endocrine Oncology, Gustave Roussy, University of Paris Sud, Villejuif, France. · Department of Gastroenterology and Pancreatology, Beaujon Hospital, University Paris 7, Clichy, France. · Department of Endocrinology, General Hospital of Chambéry, Chambéry, France. · Department of Endocrine Surgery, University Hospital of Dijon, and INSERM, U866, Epidemiology and Clinical Research in Digestive Oncology Team, and INSERM, CIC1432, Clinical Epidemiology Unit, University Hospital of Dijon, Clinical Investigation Center, Clinical Epidemiology/Clinical Trials Unit, Dijon, France. ·Ann Surg · Pubmed #28263205.

ABSTRACT: OBJECTIVE: To report long-term follow-up of patients with multiple endocrine neoplasia type 1 (MEN1) and nonfunctioning pancreatic neuroendocrine tumors (NF-PET). BACKGROUND: Pancreaticoduodenal tumors occur in almost all patients with MEN1 and are a major cause of death. The natural history and clinical outcome are poorly defined, and management is still controversial for small NF-PET. METHODS: Clinical outcome and tumor progression were analyzed in 46 patients with MEN1 with 2 cm or smaller NF-PET who did not have surgery at the time of initial diagnosis. Survival data were analyzed using the Kaplan-Meier method. RESULTS: Forty-six patients with MEN1 were followed prospectively for 10.7 ± 4.2 (mean ± standard deviation) years. One patient was lost to follow-up and 1 died from a cause unrelated to MEN1. Twenty-eight patients had stable disease and 16 showed significant progression of pancreaticoduodenal involvement, indicated by increase in size or number of tumors, development of a hypersecretion syndrome, need for surgery (7 patients), and death from metastatic NF-PET (1 patient). The mean event-free survival was 13.9 ± 1.1 years after NF-PET diagnosis. At last follow-up, none of the living patients who had undergone surgery or follow-up had evidence of metastases on imaging studies. CONCLUSIONS: Our study shows that conservative management for patients with MEN1 with NF-PET of 2 cm or smaller is associated with a low risk of disease-specific mortality. The decision to recommend surgery to prevent tumor spread should be balanced with operative mortality and morbidity, and patients should be informed about the risk-benefit ratio of conservative versus aggressive management when the NF-PET represents an intermediate risk.

4 Clinical Trial Evaluating lanreotide as maintenance therapy after first-line treatment in patients with non-resectable duodeno-pancreatic neuroendocrine tumours. 2017

Lepage, Côme / Dahan, Laetitia / Bouarioua, Nadia / Toumpanakis, Christos / Legoux, Jean-Louis / Le Malicot, Karine / Guimbaud, Rosine / Smith, Denis / Tougeron, David / Lievre, Astrid / Cadiot, Guillaume / Di Fiore, Frédéric / Bouhier-Leporrier, Karine / Hentic, Olivia / Faroux, Roger / Pavel, Marianne / Borbath, Ivan / Valle, Juan W / Rinke, Anja / Scoazec, Jean-Yves / Ducreux, Michel / Walter, Thomas. ·Department of Digestive Oncology, Burgundy Franche-Conté University, University hospital Dijon, Dijon, France; Burgundy Franche-Conté University, EPICAD, INSERM LNC UMR1231, Dijon, France; French Federation of Digestive Oncology (FFCD), INSERM LNC UMR1231 EPICAD, Dijon, France. Electronic address: come.lepage@u-bourgogne.fr. · Department of Digestive Oncology, Aix-Marseille University - Assistance Publique Hôpitaux de Marseille, Marseille, France. · Department of Gastroenterology and Digestive Oncology, Saint Etienne, France. · Royal Free Hospital, Neuroendocrine Tumour Unit, Londres, Grande-Bretagne, UK. · Regional Hospital of Orleans, Orleans, France. · French Federation of Digestive Oncology (FFCD), INSERM LNC UMR1231 EPICAD, Dijon, France. · Hôpital Rangueil, Toulouse, France. · Hôpital Haut Lévêque, Service d'hépato-gastroentérologie, Pessac, France. · Hôpital de la Milétrie, Poitiers, France. · CHU de Rennes-Hôpital Pontchaillou, Rennes, France. · Hôpital Robert Debré, Reims, France. · CHU Charles Nicolle, Rouen, France. · CHU Côte de Nacre, Caen, France. · Hôpital Beaujon, Clichy, France. · CH Les Oudairies, La Roche sur Yon, France. · Charite Campus Virchow Kinikum, Berlin, Germany. · Cliniques universitaires Saint-Luc, Bruxelles, Belgium. · University of Manchester, Division of Cancer Sciences/The Christie NHS Foundation Trust, Manchester, UK. · University Hospital Marburg, Marburg, Germany. · Pathology Department, Gustave Roussy, Villejuif, France. · Gastrointestinal Oncology Department, Gustave Roussy Institute, Villejuif, France; Faculté de Médecine, Paris Sud University Le Kremlin Bicêtre, France. · Edouard Herriot Hospital, Department of Gastroenterology, Hospices Civils de Lyon, Lyon, France. ·Dig Liver Dis · Pubmed #28292641.

ABSTRACT: INTRODUCTION: Patients with metastatic or locally advanced, non-resectable, grade 1 or 2 well-differentiated duodeno-pancreatic (WDDP) NETs are treated following European guidelines. Patients (Pts) with aggressive disease, i.e. progressive and/or symptomatic metastases and/or with significant hepatic invasion (>30-50%), and/or bone metastases, anti-tumour therapy should receive systemic combination of chemotherapy once disease control is obtained. AIM(S): The aim is to stop chemotherapy until progression. REMINET is an academic randomized, double-blind, placebo-controlled, phase II/III study designed to evaluate lanreotide (LAN) as maintenance treatment after L1 chemotherapy in G1-G2 WDDP NET. MATERIALS AND METHODS: Main eligibility criteria: adults pts with a metastatic (synchronous or metachronous) or locally advanced, non-resectable, grade 1 or 2 WDDP NETs and documented control disease after L1 therapy at least 4 weeks prior to randomization. RESULTS: 222 patients will be randomly assigned in a 1:1 ratio to receive 120mg LAN or placebo, every 28 days, until disease progression or unacceptable toxicity. The aim of the phase II part is to demonstrate a 6-months PFS >45% in LAN arm. Secondary endpoints are PFS according to central review, overall survival, safety and quality of life. A bio-bank of frozen blood will be constituted. CONCLUSION: The study is currently open in France, Germany, Belgium, United Kingdom and Ireland. A total of 25 patients are randomized (NCT02288377).

5 Clinical Trial Anti-tumour effects of lanreotide for pancreatic and intestinal neuroendocrine tumours: the CLARINET open-label extension study. 2016

Caplin, Martyn E / Pavel, Marianne / Ćwikła, Jarosław B / Phan, Alexandria T / Raderer, Markus / Sedláčková, Eva / Cadiot, Guillaume / Wolin, Edward M / Capdevila, Jaume / Wall, Lucy / Rindi, Guido / Langley, Alison / Martinez, Séverine / Gomez-Panzani, Edda / Ruszniewski, Philippe / Anonymous1290854. ·Royal Free HospitalLondon, UKCharité University Medicine BerlinBerlin, GermanyUniversity of Warmia and MazuryOlsztyn, PolandUniversity of Texas MD Anderson Cancer CenterHouston, Texas, USAUniversity HospitalVienna, AustriaDepartment of Oncology of the First Faculty of Medicine and General Teaching HospitalPrague, Czech RepublicRobert-Debré HospitalReims, FranceMarkey Cancer CenterUniversity of Kentucky, Lexington, Kentucky, USAVall d'Hebron University HospitalBarcelona, SpainWestern General HospitalEdinburgh, UKUniversità Cattolica del Sacro CuoreRome, ItalyIpsenLes Ulis, FranceIpsenBasking Ridge, New Jersey, USABeaujon HospitalClichy, FranceParis Diderot UniversityParis, France m.caplin@ucl.ac.uk. · Royal Free HospitalLondon, UKCharité University Medicine BerlinBerlin, GermanyUniversity of Warmia and MazuryOlsztyn, PolandUniversity of Texas MD Anderson Cancer CenterHouston, Texas, USAUniversity HospitalVienna, AustriaDepartment of Oncology of the First Faculty of Medicine and General Teaching HospitalPrague, Czech RepublicRobert-Debré HospitalReims, FranceMarkey Cancer CenterUniversity of Kentucky, Lexington, Kentucky, USAVall d'Hebron University HospitalBarcelona, SpainWestern General HospitalEdinburgh, UKUniversità Cattolica del Sacro CuoreRome, ItalyIpsenLes Ulis, FranceIpsenBasking Ridge, New Jersey, USABeaujon HospitalClichy, FranceParis Diderot UniversityParis, France. · Royal Free HospitalLondon, UKCharité University Medicine BerlinBerlin, GermanyUniversity of Warmia and MazuryOlsztyn, PolandUniversity of Texas MD Anderson Cancer CenterHouston, Texas, USAUniversity HospitalVienna, AustriaDepartment of Oncology of the First Faculty of Medicine and General Teaching HospitalPrague, Czech RepublicRobert-Debré HospitalReims, FranceMarkey Cancer CenterUniversity of Kentucky, Lexington, Kentucky, USAVall d'Hebron University HospitalBarcelona, SpainWestern General HospitalEdinburgh, UKUniversità Cattolica del Sacro CuoreRome, ItalyIpsenLes Ulis, FranceIpsenBasking Ridge, New Jersey, USABeaujon HospitalClichy, FranceParis Diderot UniversityParis, France Royal Free HospitalLondon, UKCharité University Medicine BerlinBerlin, GermanyUniversity of Warmia and MazuryOlsztyn, PolandUniversity of Texas MD Anderson Cancer CenterHouston, Texas, USAUniversity HospitalVienna, AustriaDepartment of Oncology of the First Faculty of Medicine and General Teaching HospitalPrague, Czech RepublicRobert-Debré HospitalReims, FranceMarkey Cancer CenterUniversity of Kentucky, Lexington, Kentucky, USAVall d'Hebron University HospitalBarcelona, SpainWestern General HospitalEdinburgh, UKUniversità Cattolica del Sacro CuoreRome, ItalyIpsenLes Ulis, FranceIpsenBasking Ridge, New Jersey, USABeaujon HospitalClichy, FranceParis Diderot UniversityParis, France. ·Endocr Relat Cancer · Pubmed #26743120.

ABSTRACT: In the CLARINET study, lanreotide Autogel (depot in USA) significantly prolonged progression-free survival (PFS) in patients with metastatic pancreatic/intestinal neuroendocrine tumours (NETs). We report long-term safety and additional efficacy data from the open-label extension (OLE). Patients with metastatic grade 1/2 (Ki-67 ≤ 10%) non-functioning NET and documented baseline tumour-progression status received lanreotide Autogel 120 mg (n = 101) or placebo (n = 103) for 96 weeks or until death/progressive disease (PD) in CLARINET study. Patients with stable disease (SD) at core study end (lanreotide/placebo) or PD (placebo only) continued or switched to lanreotide in the OLE. In total, 88 patients (previously: lanreotide, n = 41; placebo, n = 47) participated: 38% had pancreatic, 39% midgut and 23% other/unknown primary tumours. Patients continuing lanreotide reported fewer adverse events (AEs) (all and treatment-related) during OLE than core study. Placebo-to-lanreotide switch patients reported similar AE rates in OLE and core studies, except more diarrhoea was considered treatment-related in OLE (overall diarrhoea unchanged). Median lanreotide PFS (core study randomisation to PD in core/OLE; n=101) was 32.8 months (95% CI: 30.9, 68.0). A sensitivity analysis, addressing potential selection bias by assuming that patients with SD on lanreotide in the core study and not entering the OLE (n=13) had PD 24 weeks after last core assessment, found median PFS remaining consistent: 30.8 months (95% CI: 30.0, 31.3). Median time to further PD after placebo-to-lanreotide switch (n=32) was 14.0 months (10.1; not reached). This OLE study suggests long-term treatment with lanreotide Autogel 120 mg maintained favourable safety/tolerability. CLARINET OLE data also provide new evidence of lanreotide anti-tumour benefits in indolent and progressive pancreatic/intestinal NETs.

6 Clinical Trial Lanreotide in metastatic enteropancreatic neuroendocrine tumors. 2014

Caplin, Martyn E / Pavel, Marianne / Ćwikła, Jarosław B / Phan, Alexandria T / Raderer, Markus / Sedláčková, Eva / Cadiot, Guillaume / Wolin, Edward M / Capdevila, Jaume / Wall, Lucy / Rindi, Guido / Langley, Alison / Martinez, Séverine / Blumberg, Joëlle / Ruszniewski, Philippe / Anonymous1240800. ·From Royal Free Hospital, London (M.E.C.) · Charité University Medicine Berlin, Berlin (M.P.) · University of Warmia and Mazury, Olsztyn, Poland (J.B.Ć.) · University of Texas M.D. Anderson Cancer Center, Houston (A.T.P.) · University Hospital, Vienna (M.R.) · Department of Oncology of the First Faculty of Medicine and General Teaching Hospital, Prague, Czech Republic (E.S.) · Robert-Debré Hospital, Reims (G.C.), Ipsen, Les Ulis, (A.L., S.M., J.B.), Beaujon Hospital, Clichy (P.R.), and Paris Diderot University, Paris (P.R.) - all in France · Samuel Oschin Cancer Center, Cedars-Sinai Medical Center, Los Angeles (E.M.W.) · Vall d'Hebron University Hospital, Barcelona (J.C.) · Western General Hospital, Edinburgh (L.W.) · and Università Cattolica del Sacro Cuore, Rome (G.R.). ·N Engl J Med · Pubmed #25014687.

ABSTRACT: BACKGROUND: Somatostatin analogues are commonly used to treat symptoms associated with hormone hypersecretion in neuroendocrine tumors; however, data on their antitumor effects are limited. METHODS: We conducted a randomized, double-blind, placebo-controlled, multinational study of the somatostatin analogue lanreotide in patients with advanced, well-differentiated or moderately differentiated, nonfunctioning, somatostatin receptor-positive neuroendocrine tumors of grade 1 or 2 (a tumor proliferation index [on staining for the Ki-67 antigen] of <10%) and documented disease-progression status. The tumors originated in the pancreas, midgut, or hindgut or were of unknown origin. Patients were randomly assigned to receive an extended-release aqueous-gel formulation of lanreotide (Autogel [known in the United States as Depot], Ipsen) at a dose of 120 mg (101 patients) or placebo (103 patients) once every 28 days for 96 weeks. The primary end point was progression-free survival, defined as the time to disease progression (according to the Response Evaluation Criteria in Solid Tumors, version 1.0) or death. Secondary end points included overall survival, quality of life (assessed with the European Organization for Research and Treatment of Cancer questionnaires QLQ-C30 and QLQ-GI.NET21), and safety. RESULTS: Most patients (96%) had no tumor progression in the 3 to 6 months before randomization, and 33% had hepatic tumor volumes greater than 25%. Lanreotide, as compared with placebo, was associated with significantly prolonged progression-free survival (median not reached vs. median of 18.0 months, P<0.001 by the stratified log-rank test; hazard ratio for progression or death, 0.47; 95% confidence interval [CI], 0.30 to 0.73). The estimated rates of progression-free survival at 24 months were 65.1% (95% CI, 54.0 to 74.1) in the lanreotide group and 33.0% (95% CI, 23.0 to 43.3) in the placebo group. The therapeutic effect in predefined subgroups was generally consistent with that in the overall population, with the exception of small subgroups in which confidence intervals were wide. There were no significant between-group differences in quality of life or overall survival. The most common treatment-related adverse event was diarrhea (in 26% of the patients in the lanreotide group and 9% of those in the placebo group). CONCLUSIONS: Lanreotide was associated with significantly prolonged progression-free survival among patients with metastatic enteropancreatic neuroendocrine tumors of grade 1 or 2 (Ki-67 <10%). (Funded by Ipsen; CLARINET ClinicalTrials.gov number, NCT00353496; EudraCT 2005-004904-35.).

7 Clinical Trial Phase II study of first-line FOLFIRI for progressive metastatic well-differentiated pancreatic endocrine carcinoma. 2011

Brixi-Benmansour, Hedia / Jouve, Jean-Louis / Mitry, Emmanuel / Bonnetain, Franck / Landi, Bruno / Hentic, Olivia / Bedenne, Laurent / Cadiot, Guillaume. ·Service d'Hépato-Gastroentérologie et d'Oncologie Digestive, CHU de Reims, Hôpital Robert-Debré, Reims F-51092 Cedex, France. ·Dig Liver Dis · Pubmed #21831734.

ABSTRACT: BACKGROUND: Pancreatic endocrine carcinomas are rare and heterogeneous. Published results concerning treatment of advanced tumours are inconsistent and responses to standard chemotherapy remain unsatisfactory. AIM: To investigate the ability of the FOLFIRI regimen to manage progressive unresectable metastatic well-differentiated endocrine carcinomas of the pancreas as first-line chemotherapy. METHODS: 20 patients with metastatic or advanced well-differentiated endocrine carcinomas of the pancreas and progressive disease were enrolled in a prospective multicentre phase II trial to receive chemotherapy with FOLFIRI schedule (irinotecan 180mg/m(2) infusion combined with simplified LV5FU2) every 14 days. The primary end point was the non-progression rate at 6 months. RESULTS: The 6-month non-progression rate was 80% (95% confidence interval [56-94%]), with stabilisation in 15 patients and 1 objective response. Overall survival at 24 months was 65% [40-82%]. Median progression-free survival was 9.1 months [6.5-17.3 months]. The median number of administered cycles was 12 [range 1-28]. Grade 3/4 haematologic toxicity occurred in 5 patients (25%) and grade 3 digestive toxicity in 11. CONCLUSION: The FOLFIRI regimen, as first-line chemotherapy, achieved stabilisation in most patients whose tumours had been progressing and was well-tolerated. It could be an alternative therapy for advanced well-differentiated endocrine carcinomas of the pancreas.

8 Article Comparison of Temozolomide-Capecitabine to 5-Fluorouracile-Dacarbazine in 247 Patients with Advanced Digestive Neuroendocrine Tumors Using Propensity Score Analyses. 2019

de Mestier, Louis / Walter, Thomas / Brixi, Hedia / Evrard, Camille / Legoux, Jean-Louis / de Boissieu, Paul / Hentic, Olivia / Cros, Jérôme / Hammel, Pascal / Tougeron, David / Lombard-Bohas, Catherine / Rebours, Vinciane / Ruszniewski, Philippe / Cadiot, Guillaume. ·Department of Pancreatology and Gatroenterology, ENETS Centre of Excellence, Hopital Beaujon, and Paris Diderot University, Clichy, France, louis.demestier@aphp.fr. · Department of Digestive Oncology, ENETS Centre of Excellence, Edouard Herriot Hospital, Lyon, France. · Department of Hepato-Gastroenterology and Digestive Oncology and Reims-Champagne-Ardennes University, Reims, France. · Department of Medical Oncology, Poitiers University Hospital, Poitiers, France. · Department of Hepato-Gatroenterology, La Source Hospital, Orlėans, France. · Department of Epidemiology and Public Health, Le Kremlin-Bicêtre Hospital, Le Kremlin-Bicêtre, France. · Department of Pancreatology and Gatroenterology, ENETS Centre of Excellence, Hopital Beaujon, and Paris Diderot University, Clichy, France. · Department of Pathology, ENETS Centre of Excellence, Hopital Beaujon, and Paris Diderot University, Clichy, France. · Department of Digestive Oncology, ENETS Centre of Excellence, Hopital Beaujon, and Paris 7 University, Clichy, France. · Department of Gastroenterology, Poitiers University Hospital, Poitiers, France. ·Neuroendocrinology · Pubmed #30759445.

ABSTRACT: INTRODUCTION: Although chemotherapy combining 5-fluorouracil (5FU)-dacarbazine (DTIC) or temozolomide (TEM)-capecitabine (CAP) is extensively used in patients with neuroendocrine tumors (NET), they were never compared. We compared their tolerance and efficacy in advanced NET. METHODS: We evaluated the records of consecutive patients with pancreatic or small-intestine advanced NET who received 5FU-DTIC or TEM-CAP between July 2004 and December 2017 in 5 French centers. Tolerance, tumor response and progression-free survival (PFS) were compared. Factors associated with PFS were analyzed using Cox multivariate regression model. To reduce the confounding bias of the nonrandomized design, PFS was compared using propensity score analyses. RESULTS: Ninety-four (5FU-DTIC) patients and 153 (TEM-CAP) patients were included. Pancreatic NET represented 82.3% of cases and 17.1, 61.8 and 10.9% of patients had G1, G2 or G3 NET respectively. Progression at baseline was reported in 92.7% of patients with available data. Grades 3-4 adverse events occurred in 24.7 and 8.5% of TEM-CAP and 5FU-DTIC patients respectively (p = 0.002). The overall response rate was 38.3 and 39.2% respectively (p = 0.596). Median PFS on raw analysis was similar to 5FU-DTIC and TEM-CAP (13.9 vs. 18.3 months, respectively p = 0.86). TEM-CAP was associated with an increased risk of progression on the raw multivariate analysis (hazard ratio [HR] 1.90, 95% CI [1.32-2.73], p = 0.001) and when adjusted on propensity score (HR 1.65, 95% CI [1.18-2.31], p = 0.004). CONCLUSION: PFS may be longer with 5FU-DTIC than TEM-CAP in patients with advanced NET. Although patients often prefer oral chemotherapy, 5FU-DTIC is a relevant alternative. A randomized comparison is needed to confirm these results.

9 Article Chemotherapy for Well-Differentiated Pancreatic Neuroendocrine Tumours with a Ki-67 Index ≥10%: Is There a More Effective Antitumour Regimen? A Retrospective Multicentre Study of the French Group of Endocrine Tumours (GTE). 2018

Roquin, Guillaume / Baudin, Eric / Lombard-Bohas, Catherine / Cadiot, Guillaume / Dominguez, Sophie / Guimbaud, Rosine / Niccoli, Patricia / Legoux, Jean-Louis / Mitry, Emmanuel / Rohmer, Vincent / Ruszniewski, Philippe / Walter, Thomas / Ducreux, Michel / Couvelard, Anne / Scoazec, Jean-Yves / Ramond-Roquin, Aline / Caroli-Bosc, François-Xavier / Hentic, Olivia. ·Department of Hepatogastroenterology and Digestive Oncology, CHU Angers, Angers University, LUNAM University, Angers, France. ·Neuroendocrinology · Pubmed #28152531.

ABSTRACT: BACKGROUND: The best chemotherapy regimen for well- differentiated pancreatic neuroendocrine tumours (pNETs) with a Ki-67 index ≥10% is still debated. We evaluated the antitumour efficacy of various first-line chemotherapy regimens (streptozocin based, platinum based, or dacarbazine/temozolomide based) in this situation. METHODS: In this retrospective multicentre study of the French Group of Endocrine Tumours (GTE), we recruited consecutive patients with advanced well-differentiated pNETs and a Ki-67 index ≥10% receiving chemotherapy between 2000 and 2012. The primary endpoint was progression-free survival (PFS) according to RECIST. RESULTS: Seventy-four patients (42 men, median age 55.5 years) were enrolled from 10 centres. Fifty-one patients (69%) had grade 2 NET and 61 (82%) were stage IV. Median overall survival was 36.3 months. Forty-four patients (59%) received streptozocin-based, 18 (24%) platinum-based, and 12 (16%) dacarbazine/temozolomide-based chemotherapy regimens. These 3 groups were similar regarding age, functioning tumours, grade, the number of metastatic sites, and surgery for primary tumours, but not regarding surgery for metastases and time since diagnosis. Grade 3 NET (HR 2.15, 95% CI: 1.18-3.92, p = 0.012) and age above 55 years (HR 1.84, 95% CI: 1.06-3.18, p = 0.030) were associated with shorter median PFS in the multivariate analyses. Compared to streptozocin-based chemotherapy, no difference was found in terms of PFS for the platinum-based or for the dacarbazine/temozolomide-based chemotherapy regimen: median PFS was 7.2, 7.5, and 7.2 months, respectively (p = 0.51). CONCLUSIONS: Patients with intermediate or highly proliferative well-differentiated pNETs may benefit from 1 of the 3 chemotherapy regimens. Increased age and grade 3 were associated with shorter median PFS. Randomised studies searching for response predictors and the best efficacy-tolerance ratio are required to personalise the strategy.

10 Article Management of gastric neuro-endocrine tumours in a large French national cohort (GTE). 2017

Manfredi, Sylvain / Walter, Thomas / Baudin, Eric / Coriat, Romain / Ruszniewski, Philippe / Lecomte, Thierry / Laurenty, Anne-Pascale / Goichot, Bernard / Rohmer, Vincent / Roquin, Guillaume / Cojocarasu, Oana-Zvetlana / Lombard-Bohas, Catherine / Lepage, Côme / Morcet, Jeff / Cadiot, Guillaume. ·CHU Dijon, hepato-gastroenterology unit, University of Bourgogne Franche-Comté, INSERM, LNC UMR1231, F-21000, Dijon, France. sylvain.manfredi@chu-dijon.fr. · Département d'Oncologie Médicale, Hospices Civils de Lyon, Hôpital Edouard Herriot, 69437, Lyon, cedex 03, France. · Gustave Roussy, Département d'Oncologie Endocrinienne, 94805, Villejuif cedex, France. · Department of Gastroenterology and Digestive Oncology, Cochin Teaching Hospital, Paris Descartes University, Paris, France. · Beaujon Hospital and Paris Diderot University, Clichy, France. · CHRU de Tours, service d'Hépato-Gastroenterologie, CNRS, UMR 7292, GICC & Université Francois-Rabelais, Tours, France. · Department of Medical Oncology, Institut Claudius Regaud, Toulouse, France. · Department of Internal Medicine, Endocrinology and Nutrition, Hôpitaux Universitaires de Strasbourg, Faculté de Médecine, Université de Strasbourg, Strasbourg, France. · Service d'endocrinologie et maladies métaboliques, CHU d'Angers, 4 rue Larrey, 49100, Angers, France. · Service d'Hépato-Gastro-Entérologie, CHU Angers, Angers, France. · CH Le Mans, Le Mans, France. · CHU Dijon, hepato-gastroenterology unit, University of Bourgogne Franche-Comté, INSERM, LNC UMR1231, F-21000, Dijon, France. · CIC, Université de Rennes 1, Rennes, France. · Department of Hepato-Gastroenterology and Digestive Oncology, Robert-Debré University Hospital, Reims, France. ·Endocrine · Pubmed #28664309.

ABSTRACT: INTRODUCTION: Gastric neuro-endocrine tumours are rare. European guidelines for the management of neuro-endocrine tumours have been published in 2012. The aim of our survey was to study the management of gastric neuro-endocrine tumours registered in the national cohort. A prospective national cohort registers the Neuro-endocrine tumours in France since January 2003 (GTE network). We reviewed all the individual medical reports of gastric neuro-endocrine tumours in order to collect data on treatment. RESULTS: One hundred and ninety seven gastric neuro-endocrine tumours diagnosed between 1964 and 2013 in 20 centres were registered. For 181 cases data were considered complete for our survey. Eighty four tumours were type 1 (46.4%); five types 2 (2.8%); 52 types 3 (28.7%) and 40 types 4 (22.1%). Types 1 and 2 were first endoscopically managed in 93 and 60% of cases, respectively, whereas surgery was first done in 45 and 42%, respectively, of types 3 and 4. Systemic treatment, chemotherapy and/or somatostatin analogue, was first administered exclusively for types 3 and 4. Near 3% of types 1 and 40% of types 2 received at a time somatostatin analogue treatment. Five-year survival rates were 98.3, 100, 63.2 and 31.8% for types 1, 2, 3 and 4, respectively. CONCLUSION: The great majority of gastric neuro-endocrine tumours registered in this national cohort are treated in accordance with the current guidelines. The survival rates we reported must be interpreted with caution, because this cohort registered preferentially selected patients eligible for treatment. The registration of all the gastric neuro-endocrine tumours, in particular type 1 considered as benign and type 4 not eligible for specific anti-cancer treatment must be encouraged.

11 Article Poorly differentiated gastro-entero-pancreatic neuroendocrine carcinomas: Are they really heterogeneous? Insights from the FFCD-GTE national cohort. 2017

Walter, T / Tougeron, D / Baudin, E / Le Malicot, K / Lecomte, T / Malka, D / Hentic, O / Manfredi, S / Bonnet, I / Guimbaud, R / Coriat, R / Lepère, C / Desauw, C / Thirot-Bidault, A / Dahan, L / Roquin, G / Aparicio, T / Legoux, J-L / Lombard-Bohas, C / Scoazec, J-Y / Lepage, C / Cadiot, G / Anonymous2580906. ·University Hospital, Lyon, France. Electronic address: thomas.walter@chu-lyon.fr. · University Hospital, Poitiers, France. · Gustave Roussy Institute, Villejuif, France. · FFCD, Dijon, France. · Trousseau Hospital, Tours, France. · Beaujon Hospital, Clichy, France. · University Hospital, Rennes, France. · Valenciennes Hospital, Valenciennes, France. · University Hospital, Toulouse, France. · Cochin Hospital, University Paris Descartes, Paris, France. · Georges Pompidou European Hospital, University Paris-V, Paris, France. · University Hospital, Lille, France. · Hôpitalde Bicêtre, Le Kremlin Bicêtre, France. · La Timone Hospital, Marseille, France. · University Hospital, Angers, France. · Avicenne Hospital, Bobigny, France. · Hôpital de la Source, Orléans, France. · University Hospital, Lyon, France. · FFCD, Dijon, France; University Hospital, Dijon, France. · University Hospital, Reims, France. ·Eur J Cancer · Pubmed #28501762.

ABSTRACT: BACKGROUND: Diagnosis and management of poorly differentiated gastro-entero-pancreatic (GEP) neuroendocrine carcinomas (NECs) remain challenging. Recent studies suggest prognostic heterogeneity. We designed within the French Group of Endocrine Tumours a prospective cohort to gain insight in the prognostic stratification and treatment of GEP-NEC. PATIENTS AND METHODS: All patients with a diagnosis of GEP-NEC between 1st January 2010 and 31st December 2013 could be included in this national cohort. Adenoneuroendocrine tumours were excluded. RESULTS: 253 patients from 49 centres were included. Median age was 66 years. Main primary locations were pancreas (21%), colorectal (27%), oesophagus-stomach (18%); primary location was unknown in 20%. Tumours were metastatic at diagnosis in 78% of cases. Performance status (PS) at diagnosis was 0-1 in 79% of patients. Among the 147 (58%) cases reviewed by an expert pathological network, 39% were classified as small cell NEC and 61% as large cell NEC. Median Ki67 index was 75% (range, 20-100). Median overall survival was 15.6 (13.6-17.0) months. Significant adverse prognostic factors in univariate analysis were PS > 1 (hazard ratio [HR] = 2.5), metastatic disease (HR = 1.6), NSE>2 upper limit of normal [ULN]; HR = 3.2), CgA>2 ULN (HR = 1.7) and lactate dehydrogenase >2 ULN (HR = 2.1). After first-line palliative chemotherapy (CT1) with platinum-etoposide (n = 152), objective response, progression-free survival and overall survival were 50%, 6.2 and 11.6 months; they were 24%, 2.9 and 5.9, respectively, after post-CT1 FOLFIRI regimen (n = 72). CONCLUSIONS: We report a large prospective series of GEP-NEC which show the predominance of large cell type and advanced stage at diagnosis. Prognosis was found more homogeneous than previously reported, mainly impacted by PS and tumour burden.

12 Article Metachronous hormonal syndromes in patients with pancreatic neuroendocrine tumors: a case-series study. 2015

de Mestier, Louis / Hentic, Olivia / Cros, Jérôme / Walter, Thomas / Roquin, Guillaume / Brixi, Hedia / Lombard-Bohas, Catherine / Hammel, Pascal / Diebold, Marie-Danièle / Couvelard, Anne / Ruszniewski, Philippe / Cadiot, Guillaume. · ·Ann Intern Med · Pubmed #25984844.

ABSTRACT: BACKGROUND: Pancreatic neuroendocrine tumors (PNETs) may evolve and cause hormonal hypersecretion-related symptoms that were not present at the initial diagnosis, termed metachronous hormonal syndromes (MHSs). Their setting, characteristics, and outcomes are not well-described. OBJECTIVE: To describe MHSs in patients with sporadic PNETs. DESIGN: Retrospective, multicenter study. SETTING: 4 French referral centers. PATIENTS: Patients with PNETs who developed MHSs related to hypersecretion of insulin, gastrin, vasoactive intestinal peptide, or glucagon between January 2009 and January 2014. MEASUREMENTS: Tumor extension, biological markers, and treatments at initial PNET diagnosis and MHS onset. Pathologic specimens were evaluated centrally, including Ki-67 index and hormone immunolabeling. RESULTS: Of 435 patients with PNETs, 15 (3.4%) were identified as having MHSs involving the hypersecretion of insulin (5 patients), vasoactive intestinal peptide (5 patients), gastrin (2 patients), or glucagon (4 patients). Metachronous hormonal syndromes developed after a median of 55 months (range, 7 to 219) and in the context of PNET progression, stability, and tumor response in 8, 6, and 1 patients, respectively. The median Ki-67 index was 7% (range, 1% to 19%) at PNET diagnosis and 17.5% (range, 2.0% to 70.0%) at MHS onset. Immunolabeling of MHS-related peptides was retrospectively found in 8 of 14 of pathologic PNET specimens obtained before MHS diagnosis. Median survival after MHS onset was 28 months (range, 3 to 56). Seven patients with MHSs died during follow-up, all due to PNETs, including 4 patients with insulin-related MHSs. LIMITATION: Retrospective data collection and heterogeneity of pathologic specimen size and origin. CONCLUSION: Metachronous hormonal syndromes were identified more often in the context of PNET progression and increased Ki-67 indices. Patients with insulin-related MHSs may have decreased survival rates. PRIMARY FUNDING SOURCE: None.

13 Article MEN1 disease occurring before 21 years old: a 160-patient cohort study from the Groupe d'étude des Tumeurs Endocrines. 2015

Goudet, P / Dalac, A / Le Bras, M / Cardot-Bauters, C / Niccoli, P / Lévy-Bohbot, N / du Boullay, H / Bertagna, X / Ruszniewski, P / Borson-Chazot, F / Vergès, B / Sadoul, J L / Ménégaux, F / Tabarin, A / Kühn, J M / d'Anella, P / Chabre, O / Christin-Maitre, S / Cadiot, G / Binquet, C / Delemer, B. ·Centre Hospitalier Universitaire de Dijon (P.G.), Endocrine Surgery, Dijon, France · INSERM U866, Dijon, France · University of Burgundy, Dijon, France · Service d'Endocrinologie et Maladies Métaboliques (A.D., N.L-B.), Centre Hospitalier Universitaire, Hôpital Robert Debré, Reims, France, Clinique d'Endocrinologie (M.L.), Centre Hospitalier Universitaire, Nantes, France · Service de Médecine Interne et Endocrinologie (C.C-B.), Clinique Marc Linquette, Centre Hospitalier Régional et Universitaire, Lille, France · Service d'Oncologie Médicale (P.N.), Institut Paoli-Calmettes, APHM, Université Aix-Marseille, Marseille, France · Service d'Endocrinologie (H.dB.), Centre Hospitalier de Chambéry, Chambéry, France · Département d'Endocrinologie (X.B.), Hôpital Cochin, Université Paris Descartes, Paris, France · Service de Gastroentérologie-Pancréatologie (P.R.), APHP, Hôpital Beaujon et Université Paris 7 Denis Diderot, Clichy, France · Fédération d'Endocrinologie (F.B-C.), Hospice Civils de Lyon et Université Lyon 1, Groupement Hospitalier Est. Lyon, France · Service d'Endocrinologie (B.V.), Diabète et Maladies Métaboliques, Centre Hospitalier Universitaire de Dijon, Hôpital du Bocage, Dijon, France · Département d'Endocrinologie (J.L.S.), Hopital de l'Archet, Nice, France · Service de Chirurgie Générale (F.M.), Viscérale et Endocrinienne, Groupement Hospitalier Universitaire Est, Hôpital de la Pitié, Paris, France · Service d'Endocrinologie (A.T.), Centre Hospitalier Universitaire, Hôpital du Haut Levêque, Pessac, France · Département d'Endocrinologie (J.M.K.), Hôpital Universitaire de Rouen. Rouen, France · Service d'Endocrinologie (P.dA.), Centre Hospitalier d'Avignon, Avignon, France · Service d'Endocrinologie (O.C.), Diabète et Maladies Métaboliques, Centre Hospitalier Universitaire de Grenoble, Hôpital Michalon, Grenoble, France · Service d'Endocrinologie (S.C-M.), Centre Hôpitalier Universitaire. Hôpital St-Antoine, Paris, France · Service d'Hép ·J Clin Endocrinol Metab · Pubmed #25594862.

ABSTRACT: CONTEXT: Multiple endocrine neoplasia Type-1 (MEN1) in young patients is only described by case reports. OBJECTIVE: To improve the knowledge of MEN1 natural history before 21 years old. METHODS: Obtain a description of the first symptoms occurring before 21 years old (clinical symptoms, biological or imaging abnormalities), surgical outcomes related to MEN1 Neuro Endocrine Tumors (NETs) occurring in a group of 160 patients extracted from the "Groupe d'étude des Tumeurs Endocrines" MEN1 cohort. RESULTS: The first symptoms were related to hyperparathyroidism in 122 cases (75%), pituitary adenoma in 55 cases (34%), nonsecreting pancreatic tumor (NSPT) in 14 cases (9%), insulinoma in 20 cases (12%), gastrinoma in three cases (2%), malignant adrenal tumors in 2 cases (1%), and malignant thymic-NET in one case (1%). Hyperparathyrodism was the first lesion in 90 cases (56%). The first symptoms occurred before 10 years old in 22 cases (14%) and before 5 years old in five cases (3%). Surgery was performed before age 21 in 66 patients (41%) with a total of 74 operations: pituitary adenoma (n = 9, 16%), hyperparathyroidism (n = 38, 31%), gastrinoma (n = 1, 33%), NSPT (n = 5, 36%), and all cases of insulinoma, adrenal tumors, and thymic-NET. One patient died before age 21 due to a thymic-NET. Overall, lesions were malignant in four cases. CONCLUSIONS: Various MEN1 lesions occurred frequently before 21 years old, but mainly after 10 years of age. Rare, aggressive tumors may develop at any age. Hyperparathyroidism was the most frequently encountered lesion but was not always the first biological or clinical abnormality to appear during the course of MEN1.

14 Article Sunitinib achieved fast and sustained control of VIPoma symptoms. 2015

de Mestier, Louis / Walter, Thomas / Brixi, Hedia / Lombard-Bohas, Catherine / Cadiot, Guillaume. ·Department of Hepato-Gastroenterology and Digestive OncologyHôpital Robert Debré, Boulevard du Général Koenig, 51100 Reims Cedex, FranceDepartment of Gastroenterology and Digestive OncologyHospices Civils de Lyon, Hôpital Edouard Herriot, Lyon, France. · Department of Hepato-Gastroenterology and Digestive OncologyHôpital Robert Debré, Boulevard du Général Koenig, 51100 Reims Cedex, FranceDepartment of Gastroenterology and Digestive OncologyHospices Civils de Lyon, Hôpital Edouard Herriot, Lyon, France gcadiot@chu-reims.fr. ·Eur J Endocrinol · Pubmed #25305306.

ABSTRACT: VIPomas are rare-functioning neuroendocrine tumors (NETs). Overproduction of vasointestinal peptide (VIP) leads to the Verner-Morrison syndrome, whose management is challenging when refractory to somatostatin analogs. Two patients with progressive metastatic pancreatic NETs and refractory VIPoma symptoms were treated with sunitinib. This led to fast and sustained total relief of VIPoma symptoms, enabling earlier discharge from hospital and improvement in their quality of life. In both cases, sunitinib discontinuation led to the quick recurrence of watery diarrhea, which resolved within a few days after reintroducing sunitinib. The anti-secretory effect of sunitinib on VIPoma syndrome was probably not related to any anti-tumor effect. These observations agree with the rare reported cases of anti-secretory effects with targeted therapies. The sunitinib-driven inhibition of multiple-tyrosine kinase receptors might act on secretory pathways and describe sunitinib's ability to improve VIPoma symptoms. Sunitinib could be a therapeutic option to control refractory VIPoma symptoms in patients with NETs.

15 Article Long-term Prognosis of Resected Pancreatic Neuroendocrine Tumors in von Hippel-Lindau Disease Is Favorable and Not Influenced by Small Tumors Left in Place. 2015

de Mestier, Louis / Gaujoux, Sébastien / Cros, Jérôme / Hentic, Olivia / Vullierme, Marie-Pierre / Couvelard, Anne / Cadiot, Guillaume / Sauvanet, Alain / Ruszniewski, Philippe / Richard, Stéphane / Hammel, Pascal. ·*Department of Gastroenterology and Pancreatology, Beaujon Hospital, Clichy, France; †Department of Hepato-Gastroenterology and Digestive Oncology, Robert-Debré Hospital, Reims-Champagne-Ardennes University, Reims, France; ‡Department of Hepato-Pancreatico-Biliary Surgery, Beaujon Hospital, Clichy, France; §Denis-Diderot University, Paris, France; Departments of ¶Pathology, and ‖Radiology, Beaujon Hospital, Clichy, France; **Department of Pathology, Bichat Hospital, Paris, France; ††Expert National Center for Rare Cancers PREDIR, INCa/APHP, Bicêtre Hospital, Le Kremlin-Bicêtre, France; and ‡‡Oncogenetics EPHE Laboratory and INSERM U753, Faculty of Medicine Paris Sud, Le Kremlin-Bicêtre, France. ·Ann Surg · Pubmed #25185468.

ABSTRACT: BACKGROUND: Management of pancreatic neuroendocrine tumors (PNETs) associated with von Hippel-Lindau disease (VHL) is challenging because of the malignant potential and difficulty in predicting prognosis. OBJECTIVE: Compare the long-term outcome of resected VHL-PNET and sporadic PNET. METHODS: Data of all patients with VHL (n = 23) operated on for nonmetastatic PNET were reviewed. Patient characteristics and recurrence-free survival rates were compared with those in patients operated on for sporadic PNET, matched for tumor size, stage, and Ki-67 index. RESULTS: Patients in both groups had similar demographic characteristics, except that patients with VHL were younger (36 vs 56 years, P < 0.0001). Median tumor size was 30 mm. Median Ki-67 index was 3% and 4% in the VHL and sporadic groups (P = 0.95), respectively, and lymph node metastases were present in 43% and 30% of cases, respectively (P = 0.45). Sixteen (70%) patients with VHL had multiple PNET; lesions less than 15 mm were left in place in 11 patients. Median postoperative follow-up was 107 months (interquartile range, 57-124 months) and 71 months (interquartile range, 58-131 months) in the VHL and control groups, respectively. Median recurrence-free survival could not have been estimated in the VHL group due to the low number of events (hazard ratio, 5.6; 95% confidence interval, 1.4-22.6; P = 0.013). Five patients with VHL died (3 from VHL-related tumors including 1 from PNET), whereas only one control patient died due to unrelated causes. CONCLUSIONS: The long-term outcome of resected VHL-PNET is better than that of sporadic PNET. PNET less than 15 mm left in place did not progress. A parenchyma-sparing surgical strategy seems appropriate in patients with VHL-PNET, who may develop more life-threatening tumors of other organs.

16 Article [Malignant insulinoma: recommendations for workup and treatment]. 2014

Baudin, Eric / Caron, Philippe / Lombard-Bohas, Catherine / Tabarin, Antoine / Mitry, Emmanuel / Reznick, Yves / Taieb, David / Pattou, François / Goudet, Pierre / Vezzosi, Delphine / Scoazec, Jean-Yves / Cadiot, Guillaume / Borson-Chazot, Françoise / Do Cao, Christine / Anonymous1780795. ·Institut Gustave-Roussy, service de médecine nucléaire et d'oncologie endocrinienne, 94805 Villejuif cedex, France. Electronic address: eric.baudin@igr.fr. · CHU Rangueil-Larrey, pôle cardiovasculaire et métabolique, service d'endocrinologie et maladies métaboliques, 31059 Toulouse cedex 9, France. · Hôpital Édouard-Herriot, Fédération des spécialités digestives, 69003 Lyon, France. · Hôpital Haut-Lévêque, service d'endocrinologie, 33600 Pessac, France. · Institut Curie, hôpital René-Huguenin, service d'onco-gastroentérologie, 92210 Saint-Cloud, France. · CHU Côte-de-Nacre, unité fonctionnelle d'endocrinologie et maladies métaboliques, 14033 Caen cedex, France. · CHU de la Timone, service central de biophysique et de médecine nucléaire, 13005 Marseille, France. · Hôpital Claude-Huriez, service de chirurgie endocrinienne, 59000 Lille, France. · CHU de Dijon, service de chirurgie générale et endocrinienne, 21000 Dijon, France. · Institut Gustave-Roussy, service de biologie et de pathologie médicales, 94805 Villejuif cedex, France. · Hôpital Robert-Debré, service d'hépato-gastro-entérologie et de cancérologie digestive, 51100 Reims, France. · Hospices Civils de Lyon, Fédération d'endocrinologie du pole Est, Fédération d'endocrinologie et centre de médecine nucléaire, 69500 Lyon, France. · Hôpital Claude-Huriez, service d'endocrinologie et de maladies métaboliques, 59000 Lille, France. ·Presse Med · Pubmed #24857257.

ABSTRACT: Insulinoma are malignant in 4 to 14 % of cases. Their rarity and the sparse data available in the literature have limited publication of specific guidelines for their management. The following review aim to provide up-to-date recommendations on initial evaluation including pathologic grading, measures to control hypoglycemia, antitumor strategies and long term follow-up. Will be discussed in detail respective indications of surgery, diazoxide, somatostatin analogs, everolimus, sunitinib, liver directed treatments including arterial embolization, chemotherapy and radiometabolic therapy. A Medline search using terms "insulinoma", "neuroendocrine pancreatic tumors", "islet cell carcinoma", "malignant insulinoma" was performed limiting the selection to English language articles and adult age cases, along with cross referencing.

17 Article ENETS Consensus Guidelines for the management of patients with digestive neuroendocrine neoplasms: functional pancreatic endocrine tumor syndromes. 2012

Jensen, Robert T / Cadiot, Guillaume / Brandi, Maria L / de Herder, Wouter W / Kaltsas, Gregory / Komminoth, Paul / Scoazec, Jean-Yves / Salazar, Ramon / Sauvanet, Alain / Kianmanesh, Reza / Anonymous90716. ·Digestive Diseases Branch, NIH, Bethesda, MD 20892, USA. robertj@bdg10.niddk.nih.gov ·Neuroendocrinology · Pubmed #22261919.

ABSTRACT: -- No abstract --

18 Article Magnetic resonance imaging versus endoscopic ultrasonography for the detection of pancreatic tumours in multiple endocrine neoplasia type 1. 2012

Barbe, Coralie / Murat, Arnaud / Dupas, Benoit / Ruszniewski, Philippe / Tabarin, Antoine / Vullierme, Marie-Pierre / Penfornis, Alfred / Rohmer, Vincent / Baudin, Eric / Le Rhun, Marc / Gaye, Delphine / Marcus, Claude / Cadiot, Guillaume / Anonymous2780710. ·Clinical Research Coordination Unit, Robert Debré Hospital, University Hospital of Reims, Reims, France. cbarbe@chu-reims.fr ·Dig Liver Dis · Pubmed #22078814.

ABSTRACT: OBJECTIVE: In multiple endocrine neoplasia type 1, the main risk factor for metastases is pancreatic tumour size. We and others recommend limiting surgery to non-functioning pancreatic tumors ≥20 mm or growing, based on their size measured with endoscopic ultrasonography. Because endoscopic ultrasonography is invasive, we compared endoscopic ultrasonography (EUS) to non-invasive magnetic resonance imaging (MRI) for the detection of pancreatic tumours ≥10 mm in multiple endocrine neoplasia type 1 patients. METHODS: A prospective study was performed in nine participating centres; 90 patients with multiple endocrine neoplasia type 1 underwent EUS and MRI with gadolinium infusion. Gastroenterologists and radiologists were blinded to the results, magnetic resonance images were reviewed centrally. RESULTS: EUS detected 86 tumours ≥10 mm, and 48 (53.3%) patients had at least one tumour ≥10 mm. MRI detected 67 tumours ≥10 mm, and 46 (51.1%) patients had at least one tumour ≥10 mm. EUS and MRI agreement was moderate for detection of tumours ≥10 mm (Kappa coefficient=0.49), and for selection of patients with tumours ≥10 mm (Kappa coefficient=0.55). EUS and MRI missed 11/24 and 4/24 lesions ≥20 mm, respectively. EUS failed to identify 9/57 (15.7%) patients with pancreatic tumours ≥10 mm, and MRI failed to identify 11/57 (19.3%) patients with pancreatic tumours ≥10 mm. CONCLUSIONS: EUS and MRI are complementary and should be performed at initial evaluation in multiple endocrine neoplasia type 1 patients. Whether follow-up should be based on either technique or both, requires further evaluation.

19 Article Chromogranin A measurement in metastatic well-differentiated gastroenteropancreatic neuroendocrine carcinoma: screening for false positives and a prospective follow-up study. 2011

Vezzosi, Delphine / Walter, Thomas / Laplanche, Agnès / Raoul, Jean Luc / Dromain, Clarisse / Ruszniewski, Philippe / d'Herbomez, Michèle / Guigay, Joël / Mitry, Emmanuel / Cadiot, Guillaume / Leboulleux, Sophie / Lombard-Bohas, Catherine / Borson-Chazot, Françoise / Ducreux, Michel / Baudin, Eric. ·Institut Gustave Roussy, Villejuif - France. ·Int J Biol Markers · Pubmed #21574156.

ABSTRACT: BACKGROUND: Multiple causes of false-positive chromogranin A (CgA) measurement have been reported that may affect its impact as a surrogate marker of RECIST progression in well-differentiated gastroenteropancreatic neuroendocrine tumors (WDGEPNET). ? AIMS: 1) To evaluate the frequency of false-positive CgA results. 2) To prospectively compare CgA variations with RECIST morphological changes in patients without known causes of false-positive CgA measurements.? METHODS: First, the conditions responsible for potentially false-positive CgA measurements were screened in 184 consecutive patients with metastatic WDGEPNET. Secondly, a variation in CgA at a 6-month interval was compared to RECIST results at 6 months in 46 patients.? RESULTS: Among 184 patients, elevated CgA was found in 130 cases (71%) including 99 patients with at least one cause of a false-positive result. Impaired kidney function as well as medication with proton pump inhibitors were found to be the 2 major causes of false-positive results. The sensitivity and specificity of CgA measurements compared with morphological tumor changes according to the RECIST criteria were 71% and 50%, respectively, at 6 months.? CONCLUSION: Routine screening for the causes of false-positive CgA measurements is mandatory in WDGEPNET patients. Our study does not validate the use of CgA as a surrogate marker of tumor progression.

20 Article Gender-related differences in MEN1 lesion occurrence and diagnosis: a cohort study of 734 cases from the Groupe d'etude des Tumeurs Endocrines. 2011

Goudet, P / Bonithon-Kopp, C / Murat, A / Ruszniewski, P / Niccoli, P / Ménégaux, F / Chabrier, G / Borson-Chazot, F / Tabarin, A / Bouchard, P / Cadiot, G / Beckers, A / Guilhem, I / Chabre, O / Caron, P / Du Boullay, H / Verges, B / Cardot-Bauters, C. ·Centre Hospitalier Universitaire de Dijon, Service de Chirurgie Endocrinienne, Dijon, France. pierre.goudet@chu-dijon.fr ·Eur J Endocrinol · Pubmed #21551167.

ABSTRACT: CONTEXT: Multiple endocrine neoplasia type 1 (MEN1) disease is an autosomal dominant syndrome that is believed to equally affect men and women. This assumption has never been confirmed. OBJECTIVE: The aims of this study were to evaluate the impact of gender on the prevalence of MEN1 lesions, on their lifetime probability of occurrence, and on the diagnosis of MEN1. DESIGN: Data regarding a study of 734 cases of MEN1 from the multicenter 'Groupe d'étude des Tumeurs Endocrines' were analyzed. RESULTS: There were 57.8% females. The prevalence and probability of pancreatic tumors were higher in males than in females (P=0.06, P=0.0004). This difference was due to gastrinomas. The prevalence and probability of developing pituitary tumors were significantly greater in females (P<0.001, P<0.0001). Thymic tumors were exclusively found in men. There were no significant gender differences in the prevalence and the probability of developing hyperparathyroidism, or adrenal and bronchial tumors, or in the proportion of positive genetic tests. A family history of MEN1 was more frequently found in men than in women at the time of diagnosis (P=0.02). In the case of pituitary tumor, the proportion of patients diagnosed with MEN1 at the time of the first lesion was lower in women (44.2%) than in men (67.3%). CONCLUSION: The phenotype expression of the MEN1 disease gene was different in males and females. In female patients, the possibility of MEN1 is not sufficiently taken into account. Any patient presenting a lesion that belongs to the MEN1 spectrum, such as a pituitary tumor, should be closely questioned about their family history and should be tested for hypercalcemia.