Key Concepts

• Appendiceal neoplasms are rare and typically present as incidental findings upon pathologic examination following appendectomy.
• Right colectomy should be considered for appendiceal adenocarcinoma, except for tumors limited to the submucosa.
• Benign mucinous neoplasms are adequately treated by appendectomy provided resection margins are clear.
• A perforated appendiceal tumor associated with peritoneal mucin should be treated with excision of the perforated lesion, peritoneal washings for cytology, and biopsy of any suspicious peritoneal lesions. Formal cytoreductive surgery should be delayed and performed by a specialized surgeon.
• Neuroendocrine tumors smaller than 2 cm are adequately treated with appendectomy alone.
• Neuroendocrine tumors larger than 2 cm, or those having high-risk features (incomplete resection, location at the base of the appendix, Ki 67 > 2%, lymphovascular or perineural invasion, mesoappendix invasion, grade 2 or greater) should be considered for right hemicolectomy.