Neoplastic Appendicitis

Background: Appendiceal cancer as a cause of appendicitis is a rare event occurring in approximately 1.0% of all cases of appendicitis. Nevertheless, treatment of appendiceal cancer as appendicitis may have an adverse outcome for the patient, even iatrogenically converting a contained malignant process into a disseminated disease that will have a lethal outcome. Methods: Staging of appendiceal cancer and the shortcomings of the TNM are reviewed. The new standard of management for a contained or disseminated appendiceal neoplasm is presented. Results: The condition of the wall of the appendix, both before and after the resection of an appendiceal neoplasm is crucial in optimal treatment planning. The histologic grade of indolent versus aggressive disease and the extent of disease are important prognostic indicators. Cytoreductive surgery (CRS) and hyperthermic perioperative chemotherapy (HIPEC) are now standard of care if peritoneal dissemination has occurred. Judgments regarding the need for right colon resection in patients with low-grade histology have changed. Conclusions: Cancer as a cause of appendicitis is rare but since appendicitis is such a common disease process, the general surgeon and surgical oncologist must be prepared to optimally treat an appendiceal neoplasm. New paradigms for patient management have, in the recent past, become a standard of care.