Surgical therapy of pancreatic neuroendocrine neoplasms

Pancreatic neuroendocrine tumors are relatively rare entities, which consist of single or multiple benign or malignant neoplasms. It will be shown that in 10-20 % of patients, they can be associated with inherited syndromes, such as multiple endocrine neoplasia type 1. Those tumors are clinically heterogeneous and classified into functioning (10-30 %) and nonfunctioning (70-90 %) types, depending on their ability to produce symptoms due to hormone production. Because of their rarity, as well as their relatively indolent natural history, treatment approaches are not standardized, yet. A formal pancreatic resection is usually mandatory for large and localized sporadic pancreatic tumors or in the presence of symptoms. Nevertheless, in the case of small and asymptomatic lesions, a conservative approach consisting of a wait-and-see policy is going to look as more appropriate, in particular when, in order to remove the lesion, an aggressive surgical procedure is required, such as pancreaticoduodenectomy or distal splenopancreatectomy, depending on the localization of the tumor. Also, surgery plays a significant role in locally advanced and metastatic forms.