PMID- 38039101
OWN - NLM
STAT- MEDLINE
DCOM- 20231216
LR  - 20231216
IS  - 1479-683X (Electronic)
IS  - 0804-4643 (Linking)
VI  - 189
IP  - 6
DP  - 2023 Dec 6
TI  - Characteristics and treatment options of glucagonomas: a national study from the 
      French Group of Endocrine Tumors and ENDOCAN-RENATEN network.
PG  - 575-583
LID - 10.1093/ejendo/lvad157 [doi]
AB  - OBJECTIVE: 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. DESIGN AND METHODS: 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 >/= 5 kg) associated with either glucagonemia > 2 x 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). RESULTS: 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 > 3% 
      was associated with shorter OS (hazard ratio 5.27, 95% CI [1.11-24.96], P = 
      .036). CONCLUSION: 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.
CI  - (c) The Author(s) 2023. Published by Oxford University Press on behalf of European 
      Society of Endocrinology. All rights reserved. For permissions, please e-mail: 
      journals.permissions@oup.com.
FAU - Perrier, Marine
AU  - Perrier M
AUID- ORCID: 0000-0002-4528-5471
AD  - Reims-Champagne-Ardenne University, Department of Hepato-Gastroenterology and 
      Digestive Oncology, Robert Debre Hospital, Reims 51100, France.
FAU - Brugel, Mathias
AU  - Brugel M
AD  - Reims-Champagne-Ardenne University, Department of Hepato-Gastroenterology and 
      Digestive Oncology, Robert Debre Hospital, Reims 51100, France.
FAU - Gerard, Laura
AU  - Gerard L
AD  - Department of Digestive Oncology, ENETS Centre of Excellence, Hospices Civils de 
      Lyon, Lyon 69003, France.
FAU - Goichot, Bernard
AU  - Goichot B
AD  - Department of Endocrinology, Diabetology & Nutrition, Hopital Hautepierre, 
      Strasbourg University Hospital, Strasbourg 67200, France.
FAU - Lievre, Astrid
AU  - Lievre A
AD  - Department of Gastroenterology, Pontchaillou University Hospital, Rennes 
      University, INSERM U1242, Rennes 35000, France.
FAU - Lepage, Come
AU  - Lepage C
AD  - Federation Francophone de Cancerologie Digestive (FFCD), EPICAD INSERM LNC-UMR 
      1231, University of Burgundy and Franche Comte, Dijon 21000, France.
FAU - Hautefeuille, Vincent
AU  - Hautefeuille V
AD  - Department of Hepato-Gastroenterology and Digestive Oncology, Amiens University 
      Hospital, Amiens 80480, France.
FAU - Do Cao, Christine
AU  - Do Cao C
AD  - Department of Endocrinology, Hopital Claude Huriez, Lille University Hospital, 
      Lille 59000, France.
FAU - Smith, Denis
AU  - Smith D
AD  - Department of Hepato-Gastroenterology and Digestive Oncology, Haut-Leveque 
      Hospital, Bordeaux University Hospital, Pessac 33600, France.
FAU - Thuillier, Philippe
AU  - Thuillier P
AD  - Department of Endocrinology, University Hospital of Brest, Brest 29200, France.
FAU - Cros, Jerome
AU  - Cros J
AD  - Universite Paris-Cite, Department of Pathology, ENETS Centre of Excellence, 
      Beaujon Hospital (APHP.Nord), Clichy 92110, France.
FAU - Cadiot, Guillaume
AU  - Cadiot G
AD  - Reims-Champagne-Ardenne University, Department of Hepato-Gastroenterology and 
      Digestive Oncology, Robert Debre Hospital, Reims 51100, France.
FAU - Walter, Thomas
AU  - Walter T
AD  - Department of Digestive Oncology, ENETS Centre of Excellence, Hospices Civils de 
      Lyon, Lyon 69003, France.
FAU - de Mestier, Louis
AU  - de Mestier L
AD  - Universite Paris-Cite, Department of Pancreatology and Digestive Oncology, ENETS 
      Centre of Excellence, Beaujon Hospital (APHP.Nord), Clichy 92110, France.
LA  - eng
GR  - French Group of Endocrine Tumors/
GR  - IPSEN-pharma/
PT  - Journal Article
PL  - England
TA  - Eur J Endocrinol
JT  - European journal of endocrinology
JID - 9423848
RN  - 0 (Ki-67 Antigen)
SB  - IM
MH  - Humans
MH  - Middle Aged
MH  - *Glucagonoma/diagnosis/therapy/complications
MH  - Retrospective Studies
MH  - Ki-67 Antigen
MH  - *Endocrine Gland Neoplasms
MH  - *Necrolytic Migratory Erythema/complications/diagnosis/drug therapy
MH  - *Pancreatic Neoplasms/diagnosis
MH  - *Neuroendocrine Tumors/complications
MH  - *Diabetes Mellitus
MH  - Weight Loss
OTO - NOTNLM
OT  - glucagonoma
OT  - necrolytic migratory erythema
OT  - neuroendocrine neoplasms
OT  - pancreas
COIS- Conflict of interest: C.L.: Novartis, Ipsen, AMGEN, and Deciphera; V.H.: AAA, 
      Ipsen, Servier, Pierre Fabre, Deciphera, Amgen, and Merck; G.C.: AAA, Ipsen, 
      Keocyt, and Esteve; T.W.: AAA Pharma, Inc., Ipsen, ESTEVE, MSD, Pierre Fabre, and 
      TERUMO; L.d.M.: AAA, Esteve, Ipsen, and SIRTex. All other authors have no 
      conflict of interest.
EDAT- 2023/12/01 18:42
MHDA- 2023/12/17 09:46
CRDT- 2023/12/01 12:33
PHST- 2023/06/14 00:00 [received]
PHST- 2023/10/02 00:00 [revised]
PHST- 2023/10/23 00:00 [accepted]
PHST- 2023/12/17 09:46 [medline]
PHST- 2023/12/01 18:42 [pubmed]
PHST- 2023/12/01 12:33 [entrez]
AID - 7457469 [pii]
AID - 10.1093/ejendo/lvad157 [doi]
PST - ppublish
SO  - Eur J Endocrinol. 2023 Dec 6;189(6):575-583. doi: 10.1093/ejendo/lvad157.