Liver metastases of MEN1 related duodenopancreatic neuro-endocrine tumors; strategies and outcomes

Introduction: Duodenopancreatic neuroendocrine tumors (dpNETs) develop in a majority of multiple endocrine neoplasia type 1 (MEN1) patients and are the leading cause of death. Overall survival and progression-free survival for advanced dpNETs in MEN1 patients remain unknown. Methods: Cohort study using the Dutch National MEN1 database, which includes >90% of the Dutch MEN1 population between 1990-2013. Patients were classified according to their primary treatment strategy. Overall survival and progression-free survival were assessed. Results: A total of 72% of the MEN1 patients developed a dpNET of whom 34/238 (12%) developed dpNET related liver metastases. Median age at liver metastases diagnosis was 52.5 years [range 31–74]. Liver metastases histology was available in 9/34 patients (26%) showing only WHO grade 1 and 2 tumors. A total of five patients (15%) underwent locoregional therapy (LRT), 18 patients (53%) received systemic therapy (ST), five patients (15%) received a combination therapy, and six patients (17%) followed a watchful waiting strategy (WW). At the end of the median follow-up of 4 years [range 0.5-12.3] 16 patients (47%) had died. Median overall survival after diagnosis and progression-free survival after the primary treatment strategy were respectively 11.3 years (95% CI 3.5–19) and 2.0 years (95% CI 0.8–3.2). Median progression-free survival after LRT, ST, combination therapy and WW was respectively 1.0, 1.75, 2.5 and 2.8 years. Conclusions: Twelve percent of MEN1 patients with a dpNET developed liver metastases. A combination of various strategies led to a median overall survival of 11.3 years and a progression-free survival of 2 years. [Overall Survical MEN1 dpNET liver metastases]