Well-Differentiated Neuroendocrine Tumor of the Appendix: Case Report and Brief Review of the Literature

Abstract

Neuroendocrine tumors (NETs) are rare tumors detected incidentally in patients undergoing investigations for some other disease process. When occurring in the digestive system and exocrine glands, the Well-differentiated NETs are seen in the tubular structures of these organs. These are slow-growing, and many are asymptomatic. Due to the scarcity of cases, most treatment modalities are based on expert opinions and institutional guidelines. Presentation of an appendiceal well-differentiated tumor is classically an incidental finding on pathology in a patient undergoing appendectomy for presumed appendicitis. Oncological protocols are different, owing to the natural history of appendiceal neuroendocrine tumors (ANETs). This limits the accumulation of more mature data in pediatric or adult literature. ANETs need a multidisciplinary approach to handling these neoplasms. Clarifying the appendix histopathological diagnosis and classification may be complicated and require ancillary studies by the diagnostic pathologist. We present the case of a 58-year-old woman referred by her primary care physician for evaluation and surgical management of an incidentally discovered 3 cm neuroendocrine tumor in the appendix, initially thought to be appendicitis. We present this case's clinical presentation, investigation, management, and prognosis. We also discuss and review the relevant literature on this tumor.