Neuroendocrine tumors of the pancreas are rare neoplasms that may arise sporadically or in association with a hereditary endocrine neoplasia syndrome. Effective management requires directed biochemical testing, careful choice of preoperative imaging tests, and complete pancreatic exploration by an experienced endocrine surgeon utilizing intraoperative ultrasound. Pancreatic endocrine tumors arising in the familial setting present unique diagnostic and therapeutic dilemmas.
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