Introduction

The incidence of appendiceal neoplasms is 1.2 cases per 100,000 persons per year in the United States and 1-2% of appendectomies.1 Management of appendiceal neoplasms is challenging partly due to classification complexity. Following SCARE criteria 2, we present a patient with locally advanced and perforated appendiceal adenocarcinoma with no signs of peritoneal, solid organ or nodal metastases and without evidence of pseudomyxoma peritonei. To address pathologic variations and treatment algorithm inconsistencies, we also review epithelial appendiceal neoplasms based on the World Health Organization classification, current nomenclature, clinical presentations, and provide a proposed algorithm for management.