This book provides the most recent update on the management of neuroendocrine neoplasia (NEN), a term covering all tumors of various organs and/or with a particular histology, including MEN (multiple endocrine neoplasia) related tumors, MiNEN (mixed neuroendocrine-non-neuroendocrine neoplasms), NEC (neuroendocrine carcinoma) and Merkel’s carcinoma. NENs are heterogeneous in their biology, clinical presentation and prognosis, showing a great variability in aggressiveness and therapy response. As a result, their treatment is based on a large spectrum of options. The standard therapies are surgery in early disease, various loco-regional procedures in certain conditions and mostly of a palliative nature in metastatic disease. At present, thanks to our increased understanding of molecular signaling pathways, several pharmacological approaches can be used in patients with advanced NENs. Somatostatin analogs display both anti-tumor effects and symptom control. Novel peptide-radio-receptortreatment (PRRT) is used in patients with well differentiated tumors. The agents targeting angiogenesis and/or PI3K/AKT/mTOR pathway, alone or in combination with analogues, have provided encouraging results in advanced disease. The first part of the book focuses on the history, epidemiology and the most relevant scientific achievements, covering the discoveries in genetic and molecular biology, the endoscopic techniques with guided biopsy, and the metabolic imaging with hybrid PET/CT and MRI/CT. It particularly highlights the emerging strategies in therapy, surgery and mini-invasive surgery as well as loco-regional and systemic treatments, including targeted therapy and/or biological therapies. The second part then explores the management of NENs of various anatomical origins and/or with peculiar biology. It describes the range of the current options and the most relevant results from the clinical trials. This informative book provides valuable insights for all thoseinterested in the management of neuroendocrine neoplasia.