Current Status and Future Directions in Appendiceal Cancer with Peritoneal Dissemination Konstantinos I. Votanopoulos, MD, PhD, Perry Shen, MD, John H. Stewart IV, MD, Edward A. Levine, MD* INTRODUCTION Appendiceal cancer is a rare disease with an incidence in the reported literature that varies depending on the histologic types included in the classification of appendiceal malignancies. Historic evidence suggests that appendiceal primaries are diagnosed in approximately 1% of all appendectomy specimens. 1 In a Surveillance, Epidemiology and End-Results database retrospective analysis that excluded low-grade carcinoid tumors, the annual age-adjusted incidence of appendiceal primaries was 0.12 cases per 1,000,000 of population. Appendiceal adenocarcinoma represented 66.5% of these patients. 2 Extrapolating from the fact that the Surveillance, Epidemiology and End-Results program collects data from 14% of the US population, the annual inci- dence of appendiceal adenocarcinoma in the country should be around 300 to 400 Surgical Oncology Section, Department of General Surgery, Wake Forest University School of Medicine, Medical Center Boulevard, Winston-Salem, NC 27157, USA * Corresponding author. Surgical Oncology Service, Department of General Surgery, Wake Forest University School of Medicine, Medical Center Boulevard, Winston-Salem, NC 27157. E-mail address: elevine@wakehealth.edu KEYWORDS  Appendiceal cancer  Peritoneal surface disease  Hyperthermic intraperitoneal perfusion  Chemotherapy  Surgery KEY POINTS  The term “pseudomyxoma peritonei” should be used to describe a clinical sign and not a disease. It can be developed by a variety of mucin-producing primaries.  Increased volume of peritoneal disease (PCI >18) is not a contraindication for cytoreduc- tion in low-grade appendiceal primaries.  Increased volume of disease is associated with decreased survival, even in cases of complete cytoreduction. Early diagnosis and treatment is crucial.  Low-grade lesions, including DPAM, may present with positive lymph nodes. The prog- nosis of this group is decreased and similar to high-grade node-positive appendiceal primaries. Surg Oncol Clin N Am 21 (2012) 599–609 http://dx.doi.org/10.1016/j.soc.2012.07.012 surgonc.theclinics.com 1055-3207/12/$ – see front matter Ó 2012 Elsevier Inc. All rights reserved.