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.