Epithelial Neoplasms of the Appendix

Context: The appendix gives rise to an array of epithelial neoplasms showing glandular or neuroendocrine differentiation, and some tumors with elements of both cell types. Although some appendiceal neoplasms resemble their counterparts in the small and large intestines (conventional adenocarcinoma and carcinoid tumor), the appendix also gives rise to relatively unique entities including mucinous neoplasms and goblet cell carcinoid tumors, which present a challenge in pathologic classification and clinical management.

Objective: To review clinical and diagnostic issues for 3 pathologic types of epithelial neoplasms of the appendix: (1) adenocarcinoma, with specific focus on mucinous neoplasm; (2) goblet cell carcinoid tumor and associated adenocarcinoma; and (3) typical carcinoid tumor.

Data Sources: Case-derived material and literature review.

Conclusions: The most important issue in pathologic assessment of epithelial tumors of the appendix is to understand the clinical implications inherent in the diagnosis.