Introduction: Glucagonomas are rare functional pancreatic neuroendocrine tumours (p NETs), with an annual occurrence of 0.01 to 0.1 new cases per 100,000.
Objective: We focused on presenting symptoms, primary’s size and location, presence of metastases, biomarkers at diagnosis, histology, response to first line treatment (clinical and biochemical), disease fee survival (DFS) and PFS (progression free survival).
Methods: Eighteen patients were identified and reviewed retrospectively. The diagnosis was based on relevant symptoms and/or raised plasma glucagon levels (>2 times upper normal limit). Assessment of primary and metastases was based on cross-sectional and molecular imaging at diagnosis. Follow-up was complete in 14/18 (78%) of patients.
Results: Presenting symptoms included migratory necrolytic erythema (MNE) in 8 (44%), diabetes in 6 (33%), weight loss in 5 (28%) and abdominal pain in 4 (22%) patients. In 2 patients, diagnosis was based only on glucagon levels. Pancreatic primary was located at the tail (33%), head (22%), body (6%) or unrecorded (39%), whilst in 85% patients, primary size was over 3cm. Thirteen (72%) patients had liver metastases at presentation. In the majority of those patients (75%), the metastatic volume occupied < 25% of the liver. Histologically, 22% of tumours were G1, 61% were G2, 6% were G3 and grade was not available in 11% patients. In 7 patients either with localized disease or small volume hepatic metastases, surgical resection was offered, as first line treatment, and mean DFS of 67 months (range 7 - 204 months). Of the remaining 11 patients, 9 had somatostatin analogues’ (SSA) monotherapy as first line treatment, with clinical and biochemical response in > 50%, and mean PFS of 40 (range: 4 -143 months). Two patients had combination chemotherapy and SSA treatment, with PFS of 10 and 24 months, respectively.
Conclusion: Glucagonomas are large, usually grade 2 p NETs, diagnosed at a late/advanced stage of disease. MNE and diabetes are the most common presenting symptoms. SSAs represent an effective first line treatment option, for control of symptoms’ and tumour growth. Large studies are needed to identify risk factors for PFS and overall survival, as well optimal treatments’ sequence, in cases of further progression.