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Appendiceal neuroendocrine tumors (ANETs) are incidentally found in 0.2–0.7% of appendectomies for suspected appendicitis.

A 29-year-old female presented with pain in the right lower quadrant of her abdomen for the past 48 h. On emergency appendectomy for suspected acute appendicitis, a perforated appendix was found. Histopathological examination showed grade 1 (low) ANET.

Appendiceal neuroendocrine neoplasms (ANENs) are commonly found at the tip of the appendix and are treated with appendectomy alone. For few ANENs located at the base, or small tumors (≤2 cm) infiltrating the submucosa, the muscularis, the subserosa layer or the mesoappendix, right hemicolectomy is recommended. However, no guidelines have been established concerning patients with appendiceal perforation in case of ANENs.

Although rare …