General Comments

The overall prevalence of functional pancreatic endocrine tumors is low, approximately 1 to 10 per million in the population.1,2 Gastrinoma and insulinoma are the most common functional neuroendocrine tumors and account for approximately 70% to 90% of all functional pancreatic neuroendocrine tumors.2 Thus, somatostatinoma and other functional neuroendocrine tumors are rare, and no endocrine surgeon has vast experience with them. Patients with these rare tumors present with either a specific syndrome or symptoms related to the malignant nature of the tumor. Therefore, therapeutic strategies designed to treat these patients need to control the tumoral process and ameliorate the syndrome associated with it. Obviously, if the tumoral process can be completely controlled by surgical resection, the characteristic syndrome resolves. However, in many individuals with these rare tumors, the extent of the disease limits the effectiveness of surgery in completely controlling the tumor. For these patients, effective medical treatments for controlling symptoms may be available and must be considered. This chapter also describes the medical therapy that may be useful in preparing the patient for surgery or controlling symptoms in the long term.

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