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.