A Case of Familial Appendiceal Neuroendocrine Tumor

The risk of developing appendiceal neuroendocrine tumor (aNET) may be attributed to multiple factors. A familial clustering is found in less than 1% of the cases. We report the case of a 25-year-old woman who initially presented with a clinical presentation of acute appendicitis and was subsequently diagnosed with aNET by histopathological examination after an emergency appendectomy. While revealing the result to the patient, she was found to have a positive family history of appendiceal carcinoid tumor. Although rare and only found in 1% of the cases, aNET found in family history should raise the suspicion of neuroendocrine tumors in other family members.