OBJECTIVE

Glucagonomas are rare islet cell tumours, accounting for 2% of such tumours, with an annual incidence of 0.01-0.1 per million. This study aimed to describe diagnostic characteristics and treatment outcomes in patients with glucagonoma from a major referral centre.

DESIGN

A retrospective case series included patients diagnosed with glucagonoma at the ENETS Centre of Excellence, Royal Free Hospital, London, UK.

METHODS

Electronic patient records were reviewed to document baseline disease characteristics and treatment outcomes. Disease-free survival (DFS), progression-free survival (PFS), and overall survival (OS) were calculated using the Kaplan-Meier method.

RESULTS

Twenty patients (75% male, age 56.6 ± 11.6 years, mean Ki-67 index 7.3 ± 7, mean ± SD) were included; 50% had liver metastases at diagnosis. The median OS was 34 months (95% CI: 30.3-37.7). Median OS was 34, 9, and 71 months for patients with liver, lung, and skeletal metastases, respectively. At diagnosis, migratory necrolytic erythema was linked to poorer OS (22 months, 95% CI: 14.3-29.7). Median DFS following surgery was 25 months (95% CI: 3.5-46.5). For inoperable disease, Lutetium-177 (Lu)-DOTATATE peptide receptor radionuclide therapy (PRRT) demonstrated efficacy in disease control. Other treatments included somatostatin analogues, chemotherapy, and molecular-targeted agents.

CONCLUSION

Tumour grade, metastases, and NME at diagnosis influence OS in glucagonoma. Surgery is associated with the best PFS as first-line therapy, while Lu-DOTATATE PRRT effectively controls disease progression. Further studies are needed to optimise treatment sequencing for advanced glucagonoma.