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Article Dans Une Revue European Journal of Endocrinology Année : 2023

Characteristics and treatment options of glucagonomas: a national study from the French Group of Endocrine Tumors and ENDOCAN-RENATEN network

Résumé

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 one major criterion (necrolytic migratory erythema (NME) and/or recent-onset diabetes and/or weight loss ≥ 5 kg) associated with either glucagonemia >2xULN 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 glucagonomas 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 analogues (n = 6/10), respectively. All lines combined, longer TTNT was reported with chemotherapy (20.2 months). Median overall survival was 17.3 years. The Ki-67 index >3% was associated with shorter OS (HR 5.27, 95%CI [1.11-24.96], p = 0.036). CONCLUSION: Patients with glucagonoma had prolonged survival, even in the presence of metastases at diagnosis. Curative-intent surgery should always be considered. Chemotherapy, PRRT or liver-directed therapy seem to provide both substantial antitumor and antisecretory efficacy.

Dates et versions

hal-04338301 , version 1 (12-12-2023)

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Marine Perrier, Mathias Brugel, Laura Gerard, Bernard Goichot, Astrid Lièvre, et al.. Characteristics and treatment options of glucagonomas: a national study from the French Group of Endocrine Tumors and ENDOCAN-RENATEN network. European Journal of Endocrinology, 2023, European Journal of Endocrinology, 189 (6), pp.575-583. ⟨10.1093/ejendo/lvad157⟩. ⟨hal-04338301⟩
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