Surgical Evaluation of Appendiceal Neuroendocrine Tumors

Appendiceal neuroendocrine tumors (NETs) are rare with an incidence rate of 0.15–0.6 cases per 100,000 each year. The majority of appendiceal neuroendocrine tumors are diagnosed retrospectively after appendectomy that was performed for other indications. Workup includes histopathology review, biochemical laboratory studies, and cross-sectional and/or functional imaging. The extent of surgical resection, appendectomy versus right hemicolectomy with mesenteric lymphadenectomy, depends upon assessment of tumor size, location, and other high risk features. The management of advanced or metastatic disease is more complex and often requires a multidisciplinary approach but can include hepatic debulking, local ablative therapies, and/or medical therapies including somatostatin analogues, targeted therapies, and cytotoxic chemotherapies. Patients with locoregional disease have excellent survival, while patients with advanced and metastatic disease have a worse prognosis. The purpose of this text is to review the diagnosis and surgical management of well-differentiated appendiceal NETs.