Appendiceal Neoplasm

Abstract

Appendiceal neoplasms are rare and are typically found in the setting of metastatic disease or, less commonly, as an incidental finding in an appendectomy specimen in cases of appendicitis or an unrelated condition. The World Health Organization classifies appendiceal neoplasms as benign or malignant. The management of the mucinous variety of appendiceal neoplasms is influenced by the presence of peritoneal involvement. Selected patients with metastatic appendiceal neoplasms are treated with surgical cytoreduction and intraperitoneal chemotherapy and/or systemic chemotherapy. Nonmetastatic appendiceal adenocarcinoma is typically managed with staging right colectomy and adjuvant fluorouracil-based chemotherapy based on colon cancer guidelines. Neuroendocrine tumors (NETs) constitute 50 to 77% of all appendiceal neoplasms and 19% of all gastrointestinal NETs. Right hemicolectomy is indicated if the size of the NET is more than 2 cm, the base of the appendix is involved, it is a high-grade tumor, or the depth of invasion of the mesoappendix is more than 3 mm. This review contains 7 figures, 9 tables and 37 references Key Words: appendiceal adenocarcinoma, appendiceal adenoma, appendiceal neoplasm, diffuse peritoneal adenomucinosis, Goblet cell carcinoid of appendix, low-grade appendiceal mucinous neoplasm, mucocele of appendix, neuroendocrine tumor of appendix, peritoneal mucinous carcinomatosis (PMCA), pseudomyxoma peritonei