ECE2023 Poster Presentations Pituitary and Neuroendocrinology (123 abstracts)
Guys and St Thomas NHS Foundation Trust, London, United Kingdom
Introduction: Pancreatic neuroendocrine tumours (pNETs) are the main cause of mortality in patients with multiple endocrine neoplasia type 1 (MEN1). The CLARINET study demonstrated that somatostatin analogue treatment improved progression free survival in patients with enteropancreatic NETs but little is known about the role of SSA in preventing progression of pNETs in MEN1 (1). Many centres have started using SSA treatment in MEN1 patients with enlarging pNETs or tumours >15 mm. This is current practice in our centre.
Objective: This observational study examined the effectiveness of somatostatin analogue (SSA) treatment in preventing progression of pNETS in MEN1.
Methods: Using our database of MEN1 patients, electronic patient records were individually reviewed to document the radiological, biochemical and clinical behaviour of pNETs over time. Patients were treated with SSA if tumours had documented growth, were > 15 mm in size or were associated with metastases. We compared pNET size/ growth in patients treated and untreated with SSA (overwhelmingly lanreotide autogel 120 mg 4 weekly).
Results:
Total MEN1 cohort | |
n | 57 |
Gender | 37F: 20M |
Age (y) | 45 (16) |
PNETs documented | 44 (12 surgical) |
Deceased (n) | 8 |
Mean age of death, y (range) | 59 (41-84) |
SSA use (%) | 35.1 |
Metastases N (%) | 4 (7.02) |
Surgical intervention? N (%) | 16 (28.1) |
Other Rx (n) | Peptide receptor radiotherapy (PRRT) (n=2) |
MEN1 cohort with pNETs on SSA | MEN1 cohort with pNETs not on SSA | |
n | 20 | 12 |
Gender | 12F: 8M | 8F:4M |
Age (y) | 42.2 (12.9) | 41.6 (17.6) |
Deceased (n) | 3 | 2 |
Mean age of death (y) | 56 | 51 |
Cause of deaths | Metastatic pNET x2, metastatic gastrinoma | ACC, metastatic pNET |
Surgical intervention? (n) | 3 | 1 |
PNET size (mm)(SD) | 15 (8) | 8 (4) |
Stable disease | 11 | 8 |
Progression | 1 | 1 |
Improvement | 4 | 0 |
Data unavailable | 4 | 3 |
Duration of SSA use | 4- 120 (mean: 48)(SD 24) | N/A |
Duration of total surveillance (mths) | 61.9 (25.2) | 34.8 (24.6) |
SEs reported | Minimal reporting-GI Symptoms, GB sludge, ?pancreatitis | N/A |
Other Rx | 1 patient had PRRT |
Conclusion: These data indicate that mean duration of 4 years SSA treatment is associated with stable size or reduction (20%) in pNETs averaging 1.5 cm in patients with MEN1. Multi-centre collaboration, including comparison of a larger untreated cohort would clarify the effectiveness of SSA preventing progression on pNETs in MEN1.
Reference: Caplin ME, et al. Lanreotide in metastatic enteropancreatic neuroendocrine tumors. N Engl J Med. 2014;371:224233.