Preoperative Imaging Overestimates the Tumor Size in Pancreatic Neuroendocrine Neoplasms Associated with Multiple Endocrine Neoplasia Type 1

Background Radiological tumor size of non-functioning pancreatic neuroendocrine neoplasms (Nf-pNENs) associated with multiple endocrine neoplasia type 1 (MEN1) is a crucial parameter to indicate surgery. The aim of this study was to compare radiological size (RS) and pathologic size (PS) of MEN1 associated with pNENs. Methods Prospectively collected data of MEN1 patients who underwent pancreatic resections for pNENs were retrospectively analyzed. RS was defined as the largest tumor diameter measured on endoscopic ultrasound (EUS), magnetic resonance imaging (MRI) or computed tomography (CT). PS was defined as the largest tumor diameter on pathological analysis. Student’s t test and linear regression analysis were used to compare the median RS and PS. p < 0.05 was considered significant. Results Forty-four patients with a median age of 37 (range 10–68) years underwent primary pancreatic resections for pNENs. Overall, the median RS (20 mm, range 3–100 mm) was significantly larger than the PS (13 mm, range 4–110 mm) ( p = 0.001). In patients with pNENs < 20 mm ( n = 27), the size difference (median RS 15 mm vs PS 12 mm) was also significant ( p = 0.003). However, the only modality that significantly overestimated the PS was EUS (median RS 14 mm vs 11 mm; p = 0.0002). RS overestimated the PS in 21 patients (21 of 27 patients, 78%). Five of 11 patients (12%) with a Nf-pNEN and a RS > 20 mm had in reality a PS < 20 mm. MRI was the imaging technique that best correlated with PS in the total cohort ( r = 0.8; p < 0.0001), whereas EUS was the best correlating imaging tool in pNENs < 20 mm ( r = 0.5; p = 0.0001). Conclusion Preoperative imaging, especially EUS, frequently overestimates the size of MEN1-pNENs, especially those with a PS < 20 mm. This should be considered when indicating surgery in MEN1 patients with small Nf-pNENs.