Surgical and Oncologic Outcomes After Pancreatectomy for Pancreatic Neuroendocrine Tumor in Multiple Endocrine Neoplasia Type 1 and von Hippel-Lindau Syndrome: A Large, Multi-Institutional, Cohort Study.

Abstract

Pancreatic neuroendocrine tumors (PNETs) are associated with MEN type 1 (MEN1) and von Hippel-Lindau (VHL) syndrome, yet data about surgical outcomes in these patients is lacking. This study compared outcomes after pancreatectomy across a large cohort of sporadic, MEN1-, and VHL-PNET patients. Retrospective analysis of pancreatectomies for PNETs performed at 5 institutions in the US between 2000 and 2023 as part of the PAncreatic Neuroendocrine Disease Alliance. All adult patients with MEN1- and VHL-associated PNETs were included. Adult sporadic PNET patients with overall survival and tumor grade data were included. Of 1,527 patients, 73 had MEN1, 26 had VHL, and 1,428 had sporadic PNETs. Majority were male (54%) with median age of 59 years (interquartile range 50 to 68 years). Median follow-up was 53 months. MEN1 and VHL patients were significantly younger and had more pancreatic head and multifocal disease compared with sporadic patients. MEN1 patients had more functional tumors (19.2% vs 10.9% sporadic vs 3.8% VHL, p= 0.045). Formal resection was most common, whereas enucleation occurred in 9.4%, 6.8%, and 3.8% of sporadic, MEN1-, and VHL-PNETs, respectively. Tumors were predominantly grade 1/2 (97%) but larger in MEN1 (2.8 vs 2.3 cm sporadic vs 2.5 cm VHL, p = 0.03). R2 resection occurred in 2.6% of sporadic, 9.6% of MEN1-, and 3.8% of VHL-PNETs. Rates of lymphadenectomy (86% to 92%), positive lymph nodes (24% to 31%), and Clavien-Dindo grade ≥3 complications were similar across groups. Progression-free survival and overall survival among groups were not significantly different. Despite significant differences in age, tumor size, R status, and surgical approach, outcomes including postoperative complications, progression-free survival and overall survival after surgery are similar between sporadic, MEN1-, and VHL-associated PNET patients at 5-year follow-up.