Tailor-made treatment of patients with multiple endocrine neoplasia type 1-related duodenopancreatic neuroendocrine tumors

Multiple endocrine neoplasia type 1 (MEN) is a rare tumor syndrome leading to multiple neuroendocrine tumors in the duodenum and pancreas already at a young age. These tumors are the leading cause of death and can secrete multiple hormones leading to clinical symptoms. The screening program aims at early diagnosis and subsequent timely interventions of which surgical resection is the only potential curative therapy. However, at present, many diagnostic challenges arise and determining the timing and extent of operative resection is a risk-benefits balance analysis guided by the oncological benefits against the risks of potential complications and long-term side effects. Therefore, this thesis aims to provide insights in the diagnosis, prognosis and surgical therapy of patients with MEN1-related duodenopancreatic neuroendocrine tumors. The findings presented in this thesis will provide a basis for the tailor-made treatment of patients with MEN1-related duodenopancreatic neuroendocrine tumors.