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SUMMARY OBJECTIVE: This study aimed to reveal the incidence, clinicopathological, and oncological outcomes of appendiceal neoplasms. METHODS: This is a retrospective cohort study from a single institution. Patients with a pathological diagnosis of malignancy who underwent appendectomy between January 2011 and 2021 were included in the study, and groups were formed according to pathological type. Clinical, pathological, and oncological results were compared in these groups. RESULTS: The incidence of neoplasia was 2.38% (n=34) in a cohort of 1,423 appendectomy cases. Of the cases, 56% (n=19) were female. The median age in the entire cohort was 55.5 (range: 13–106) years. In the cohort, the rate of neuroendocrine tumor mucinous cystadenoma adenocarcinoma, and low-grade appendiceal mucinous neoplasm, according to the American Joint Committee on Cancer classification of appendiceal neoplasms, was 32.3% (n=11), 26.4% (n=9), 26.4% (n=9), and 14.7% (n=5), respectively. Neuroendocrine tumor patients (median age: 35 years) were younger than the other groups (p=0.021). Secondary complementary surgery was performed in 66.7% (n=6) of adenocarcinoma patients and 27.3% (n=3) of neuroendocrine tumor patients. Right hemicolectomy was performed in all neuroendocrine tumor patients requiring secondary surgery, while right hemicolectomy was performed in three adenocarcinoma patients and cytoreductive surgery and hyperthermic intraperitoneal chemotherapy in three adenocarcinoma patients. After a median follow-up of 44.4 months (95% confidence interval: 18.6–70.1), the mean survival rate was 55% in appendiceal adenocarcinoma patients compared to 100% in neuroendocrine tumor patients. CONCLUSION: Appendiceal neoplasms are rare but remain an important cause of mortality. Appendiceal adenocarcinomas are associated with poorer oncological outcomes compared to other neoplasms.