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

Background: 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.

Study design: 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.

Results: 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.

Conclusions: 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.