Appendix cancer classification

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Soroush Seifirad, M.D.[2]

Overview

• Appendix cancer is classified according to the histological findings. According to WHO classification there are four major groups of appendix cancer including epithelial tumors, non-epithelial tumors, secondary tumors, and hyperplastic (metaplastic) polyps. Carcinoid (well differentiated endocrine neoplasm), and adenocarcinoma are two major subtypes of epithelial tumors, making the majority of appendix cancer cases.

Classification

• Appendix cancer may be classified according to WHO classification into 4 groups:

• Epithelial tumors
• Non-epithelial tumors
• Secondary tumors
• Hyperplastic (metaplastic) polyps.

• The table below summarizes the different types of appendix cancer according to the WHO classification.

WHO histological classification Tumors of the appendix (Adapted from WHO/IARC)
Epithelial tumors	Non-epithelial tumors	Secondary tumors	Hyperplastic polyp
Adenoma Tubular Villous Tubulovillous Serrated Carcinoma Adenocarcinoma Mucinous adenocarcinoma Signet-ring cell carcinoma Small cell carcinoma Undifferentiated carcinoma Carcinoid (well differentiated endocrine neoplasm) EC-cell, serotonin-producing neoplasm L-cell, glucagon-like peptide and PP/PYY producing tumour others Tubular carcinoid Goblet cell carcinoid (mucinous carcinoid) Mixed carcinoid-adenocarcinoma Others	Neuroma Lipoma Leiomyoma Gastrointestinal stromal tumor Leiomyosarcoma Kaposi sarcoma Others Malignant lymphoma

• Goblet cell carcinoids : Also called adenocarcinoids since they have features of both adenocarcinoma as well as carcinoid tumors. Prognosis is better than adenocarcinomas but worse than carcinoids.

• Appendical mucucele : Prognosis relate to histologic subtype

• Mucosal hyperplasia

• Simple or retention cyst

• Mucinous cystadenoma

• Mucinous cystadenocarcinoma: worst prognosis

• Appendix adenocarcinoma has been also classified as follows:

• Mucinous type adenocarcinoma : (most common): most favorable prognosis

• Intestinal or colonic adenocarcinoma :(less common): similar to colon adenocarcinoma

• Signet ring cell adenocarcinoma : (least common), poor prognosis