{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,29]],"date-time":"2026-10-29T18:29:01Z","timestamp":1793298541313,"version":"4.5.0"},"reference-count":25,"publisher":"Oxford University Press (OUP)","issue":"6","license":[{"start":{"date-parts":[[2023,12,1]],"date-time":"2023-12-01T00:00:00Z","timestamp":1701388800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"funder":[{"name":"French Group of Endocrine Tumors"},{"DOI":"10.13039\/501100014382","name":"IPSEN-pharma","doi-asserted-by":"crossref","id":[{"id":"10.13039\/501100014382","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2023,12,6]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:sec>\n                  <jats:title>Objective<\/jats:title>\n                  <jats:p>Glucagonoma is a very rare functional pancreatic neuroendocrine tumor (PanNET). We aimed to provide data on the diagnosis, prognosis, and management of patients with glucagonoma.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Design and Methods<\/jats:title>\n                  <jats:p>In this retrospective national cohort, we included all patients with glucagonoma, defined by at least 1 major criterion (necrolytic migratory erythema [NME] and\/or recent-onset diabetes, and\/or weight loss \u2265 5\u2005kg) associated with either glucagonemia &amp;gt; 2 \u00d7 upper limit of normal or positive glucagon immunostaining. Antisecretory efficacy was defined as partial\/complete resolution of glucagonoma symptoms. Antitumor efficacy was assessed according to the time to next treatment (TTNT).<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Results<\/jats:title>\n                  <jats:p>Thirty-eight patients were included with median age 58.7 yo, primary PanNET located in the tail (68.4%), synchronous metastases (63.2%). Median Ki-67 index was 3%. Most frequent glucagonoma symptoms at diagnosis were NME (86.8%), weight loss (68.4%), and diabetes (50%). Surgery of the primary PanNET was performed in 76.3% of cases, mainly with curative intent (61.5%). After surgery, complete resolution of NME was seen in 93.8% (n = 15\/16). The secretory response rates were 85.7%, 85.7%, 75%, and 60% with surgery of metastases (n = 6\/7), chemotherapy (n = 6\/7), liver-directed therapy (n = 6\/8), and somatostatin analogs (n = 6\/10), respectively. All lines combined, longer TTNT was reported with chemotherapy (20.2 months). Median overall survival (OS) was 17.3 years. The Ki-67 index &amp;gt; 3% was associated with shorter OS (hazard ratio 5.27, 95% CI [1.11-24.96], P = .036).<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Conclusion<\/jats:title>\n                  <jats:p>Patients with glucagonoma had prolonged survival, even in the presence of metastases at diagnosis. Curative-intent surgery should always be considered. Chemotherapy, peptide receptor radionuclide therapy, or liver-directed therapy seems to provide both substantial antitumor and antisecretory efficacies.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1093\/ejendo\/lvad157","type":"journal-article","created":{"date-parts":[[2023,11,30]],"date-time":"2023-11-30T06:27:15Z","timestamp":1701325635000},"page":"575-583","source":"Crossref","is-referenced-by-count":13,"title":["Characteristics and treatment options of glucagonomas: a national study from the French Group of Endocrine Tumors and ENDOCAN-RENATEN network"],"prefix":"10.1093","volume":"189","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4528-5471","authenticated-orcid":false,"given":"Marine","family":"Perrier","sequence":"first","affiliation":[{"name":"Reims-Champagne-Ardenne University, Department of Hepato-Gastroenterology and Digestive Oncology, Robert Debr\u00e9 Hospital , Reims 51100 , France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mathias","family":"Brugel","sequence":"additional","affiliation":[{"name":"Reims-Champagne-Ardenne University, Department of Hepato-Gastroenterology and Digestive Oncology, Robert Debr\u00e9 Hospital , Reims 51100 , France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laura","family":"G\u00e9rard","sequence":"additional","affiliation":[{"name":"Department of Digestive Oncology, ENETS Centre of Excellence, Hospices Civils de Lyon , Lyon 69003 , France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bernard","family":"Goichot","sequence":"additional","affiliation":[{"name":"Department of Endocrinology, Diabetology & Nutrition, H\u00f4pital Hautepierre, Strasbourg University Hospital, Strasbourg 67200 , France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Astrid","family":"Li\u00e8vre","sequence":"additional","affiliation":[{"name":"Department of Gastroenterology, Pontchaillou University Hospital, Rennes University, INSERM U1242 , Rennes 35000 , France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Come","family":"Lepage","sequence":"additional","affiliation":[{"name":"Federation Francophone de Canc\u00e9rologie Digestive (FFCD), EPICAD INSERM LNC-UMR 1231, University of Burgundy and Franche Comt\u00e9 , Dijon 21000 , France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vincent","family":"Hautefeuille","sequence":"additional","affiliation":[{"name":"Department of Hepato-Gastroenterology and Digestive Oncology, Amiens University Hospital , Amiens 80480 , France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christine","family":"Do Cao","sequence":"additional","affiliation":[{"name":"Department of Endocrinology, H\u00f4pital Claude Huriez, Lille University Hospital , Lille 59000 , France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Denis","family":"Smith","sequence":"additional","affiliation":[{"name":"Department of Hepato-Gastroenterology and Digestive Oncology, Haut-L\u00e9v\u00eaque Hospital, Bordeaux University Hospital , Pessac 33600 , France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Philippe","family":"Thuillier","sequence":"additional","affiliation":[{"name":"Department of Endocrinology, University Hospital of Brest , Brest 29200 , France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J\u00e9r\u00f4me","family":"Cros","sequence":"additional","affiliation":[{"name":"Universit\u00e9 Paris-Cit\u00e9, Department of Pathology, ENETS Centre of Excellence, Beaujon Hospital (APHP.Nord) , Clichy 92110 , France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Guillaume","family":"Cadiot","sequence":"additional","affiliation":[{"name":"Reims-Champagne-Ardenne University, Department of Hepato-Gastroenterology and Digestive Oncology, Robert Debr\u00e9 Hospital , Reims 51100 , France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas","family":"Walter","sequence":"additional","affiliation":[{"name":"Department of Digestive Oncology, ENETS Centre of Excellence, Hospices Civils de Lyon , Lyon 69003 , France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Louis","family":"de Mestier","sequence":"additional","affiliation":[{"name":"Universit\u00e9 Paris-Cit\u00e9, Department of Pancreatology and Digestive Oncology, ENETS Centre of Excellence, Beaujon Hospital (APHP.Nord) , Clichy 92110 , France"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2023,12,1]]},"reference":[{"issue":"2","key":"2023122114290371700_lvad157-B1","doi-asserted-by":"crossref","first-page":"98","DOI":"10.1159\/000335591","article-title":"ENETS consensus guidelines for the management of patients with digestive 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