Studying Prognosis and Treatment in Patients with Neuroendocrine Neoplasia

Abstract

Neuroendocrine neoplasia (NEN) are often referred to as a homogeneous group of malignancies, especially gastroenteropancreatic neuroendocrine tumours (GEP-NETs). Although they might appear similar histologically, they differ in hormonal production, molecular profile, and clinical aggressiveness based on anatomical location. These differences have important implications for prognosis and treatment. Unfortunately, until recently their heterogeneity has not been fully recognized and literature often focussed on NEN or GEP-NETs as a whole or in case of Merkel cell carcinoma (MCC) within a group of rare malignancies of the skin, hampering research and the understanding of these malignancies. The Netherlands Cancer Institute and UMC Utrecht form an European Neuroendocrine Tumour Society (ENETS) Centre of Excellence specialized in treating patients with NEN. Because of this collaboration patients can benefit from an experienced multidisciplinary team providing them with all the current relevant treatment options. Furthermore, it provides research opportunities due to concentrated care in a relatively large patient population with detailed information on treatment and survival. More understanding of prognostic factors and treatment effects could improve clinical outcome for individual patients with NEN. This thesis aims to analyse prognosis, possible biomarkers and various treatments in patients with a SI-NET and MCC. Unique for this research project is the active involvement of the Dutch NET and NEC patient Advocacy Group (Stichting NETgroep).