Bronchopulmonary neuroendocrine tumors (bpNET) - A comparison of three subgroups

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Introduction: bpNET are a rare malignancy of the lung, that arise sporadically (sp-bpNET), in the context of Multiple Endocrine Neoplasia Type 1 (MEN1) syndrome, or in the context of Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH). Until now, bpNET of any type are regarded as similar entities. Clinically however, bpNET within MEN1 or DIPNECH rarely metastasize or lead to bpNET related death.

Aim(s): This study is the first to describe and compare the disease course of bpNET in these three contexts.

Materials and methods: All patients in the DutchMEN Study Group database (1990-2017) with MEN1 and histopathologically confirmed bpNET were included. All patients with surgically resected sp-bpNET and all DIPNECH patients referred to a Dutch ENETS center between 2000-2018 were included. Chi-square test was used for comparison between groups. Kaplan-Meier and logrank test were used to compare disease specific survival (DSS) between groups. Cox regression was performed to identify predictors of DSS in sp-bpNET patients.

Conference: 18th Annual ENETS Concerence (2021)

Presenting Author: Levy S

Authors: Levy S, van den Broek M, Dijke K, Hartemink K, van Leeuwaarde R,

Keywords: bpNET, MEN1, DIPNECH,