Endocrine Abstracts

Introduction: Pancreatic neuroendocrine tumours (pNETs) can either occur in patients with a familial syndrome, like multiple endocrine neoplasia type 1 (MEN-1), or being sporadic. In the last decade, endoscopic ultrasound (EUS) has become one of the first-line investigations for the characterisation of pNETs. The ultrasonographic features of a pNET might differ depending on the familial vs sporadic pathogenesis of the tumour. Therefore, the EUS findings might help and direct the accurate definition of a pNET with a possible impact on the most appropriate diagnostic and therapeutic management of pNET patients.

Patients and methods: In this single-centre retrospective study, we have reviewed the EUS characteristics of 131 pNETs from 38 MEN-1 patients and 14 pNETs from 13 sporadic disease patients at the time of their first EUS assessment. The patients attended consecutively our institution over a 5-year-time period. With the goal of defining the EUS features of MEN-1 vs sporadic pNETs, we have analysed the most relevant morphological and ultrasonographic aspects of the tumours and compared the findings between the two patient groups.

Results: Patients with MEN-1 are more likely to present with multiple (3.4 vs 1.1 tumours/patient) and bigger (21.6 vs 7.5 mm) pNETs in comparison to those with sporadic disease. There is no statistical difference with regard to morphology, definition of the margins, and vascularization of the pNETs between the two groups. However, pNETs appear to be significantly more heterogeneous in patients with MEN-1 than in those with sporadic disease.

Conclusion: In patients with MEN-1 pNETs tend to be more numerous, bigger, and more heterogeneous than in patients with sporadic disease. EUS can help with the precise characterization of a pNET, including the definition of ultrasonographic features which can distinguish a familial vs sporadic disease.