Abstract
IntroductionUntil now, well-differentiated bronchopulmonary neuroendocrine tumors (bpNET) occurring either sporadically (sp-bpNET) or in the context of multiple endocrine neoplasia type 1 (MEN1) and diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) are regarded as similar entities. However, in contrast to sp-bpNET: MEN1-related and DIPNECH-related bpNET rarely metastasize or lead to bpNET-related death. We aimed to describe and compare the course of the disease of sp-bpNET, DIPNECH- and MEN1-related bpNET. MethodsAll patients with histologically confirmed MEN1-related bpNET from the DutchMEN Study Group database (1990-2017), patients with resected sp-bpNET and DIPNECH patients referred to a Dutch European Neuroendocrine Tumor Society center between 2000 and 2018 were included. Fisher’s exact test was used for comparison between groups. The primary end point was disease-specific mortality (DSM). Kaplan-Meier and logrank test were used to compare survival. Cox regression was used to identify risk factors for DSM in the sp-bpNET subgroup. ResultsWe included 112 sp-bpNET, 29 MEN1, and 27 DIPNECH patients. Tumor classification was similar across subgroups. A total of 20 patients (18%) with sp-bpNET died because of bpNET, compared with none in the MEN1 group and DIPNECH group. Median disease-specific survival was 12.3 (confidence interval: 6.3–18.3) years for patients with sp-bpNET, and not estimable for the other subgroups (p < 0.001). Differences in baseline characteristics did not explain worse survival in sp-bpNET. Tumor classification and age at diagnosis were independent risk factors for DSM in sp-bpNET. ConclusionsPatients with sp-bpNET have a significantly higher DSM compared with MEN1 or DIPNECH-related bpNET, unexplained by differences in baseline characteristics. This implies that not all bpNET are similar entities.
Highlights
Until now, well-differentiated bronchopulmonary neuroendocrine tumors occurring either sporadically or in the context of multiple endocrine neoplasia type 1 (MEN1) and diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) are regarded as similar entities
These neoplasms can be classified as bronchopulmonary neuroendocrine tumors, with a subdivision in typical carcinoid (TC) and atypical carcinoid (AC); SCLC or large cell neuroendocrine carcinoma (LCNEC)
Study Design and Patients All patients with sp-bronchopulmonary neuroendocrine tumors (bpNET) referred to the Netherlands Cancer Institute (NKI) and University Medical Center Utrecht (UMCU) European Neuroendocrine Tumor Society Center of Excellence (ENETS CoE) between 2000 and 2019 who had undergone surgery with curative intent were included
Summary
Well-differentiated bronchopulmonary neuroendocrine tumors (bpNET) occurring either sporadically (sp-bpNET) or in the context of multiple endocrine neoplasia type 1 (MEN1) and diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) are regarded as similar entities. These neoplasms can be classified as bronchopulmonary neuroendocrine tumors (bpNET), with a subdivision in typical carcinoid (TC) and atypical carcinoid (AC); SCLC or large cell neuroendocrine carcinoma (LCNEC) All these tumors have been grouped under “bpNET” in the most recent WHO Classification of Lung Tumors in 2015.1 Classification is based on histopathological features, including mitotic count, the presence or absence of necrosis and a variety of cytologic and morphologic features.[1] TCs and ACs— historically called “carcinoid”—account for 1% to 2% of all lung malignancies and are considered welldifferentiated tumors with an overall favorable course.[2] grouped together with the poorly differentiated SCLC and LCNEC, the 2015 WHO classification recognizes the evident major clinical, epidemiologic, histologic and genetic differences between lung carcinoids and the high-grade SCLC and LCNEC.[1] For the purpose of this article, we consider only the well-differentiated typical and ACs of the lung, which we will refer to as bpNET. Another context in which bpNET may arise, is diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH), a proliferation of neuroendocrine cells
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