Well-Differentiated Bronchopulmonary Neuroendocrine Tumors: More Than One Entity
| Autor: | Gerlof D. Valk, Margot E T Tesselaar, Wouter W. de Herder, Peter H. Bisschop, Wieneke A. Buikhuisen, Bas Havekes, Kim Dijke, Olaf M. Dekkers, Wouter T Zandee, Medard F M van den Broek, Sonja Levy, Rachel S van Leeuwaarde, Koen J. Hartemink, Menno R. Vriens, Madeleine L. Drent, Annenienke C van de Ven |
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| Přispěvatelé: | Endocrinology, AMS - Ageing & Vitality, AMS - Musculoskeletal Health, AGEM - Amsterdam Gastroenterology Endocrinology Metabolism |
| Jazyk: | angličtina |
| Rok vydání: | 2021 |
| Předmět: |
Lung Diseases
Pulmonary and Respiratory Medicine medicine.medical_specialty Lung Neoplasms Survival Context (language use) Neuroendocrine tumors Multiple endocrine neoplasia type 1 (MEN1) Gastroenterology Internal medicine Humans Medicine MEN1 Multiple endocrine neoplasia Bronchopulmonary neuroendocrine tumor (NET) business.industry Proportional hazards model Hyperplasia medicine.disease Sporadic Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) Log-rank test Neuroendocrine Tumors Exact test Oncology business Precancerous Conditions |
| Zdroj: | Journal of thoracic oncology, 16(11), 1810-1820. International Association for the Study of Lung Cancer |
| ISSN: | 1556-0864 |
| Popis: | Introduction: Until 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. Methods: All 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. Results: We 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. Conclusions: Patients 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. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
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