Miscellaneous Rare Malignancies: Pseudomyxoma Peritonei

Pseudomyxoma peritonei (PMP) is a relatively uncommon disease representing mucinous ascites, peritoneal implants, and omental caking often from ruptured appendiceal mucinous neoplasms (AMNs). Acute care surgeons often encounter PMP due to a broad range of presentations such as appendicitis, hernia, ascites, ovarian mass, and bowel obstruction. Therefore, it is essential to understand the wide spectrum of neoplasms that are grouped within AMNs and the influence of histopathology on management decisions. This chapter describes the current categorization of AMNs and PMP, clinical presentations, and initial management for general surgeons. Although general surgeons may facilitate the initial workup and acute management of symptoms of AMNs, patients should ultimately be referred to a peritoneal malignancy center for further evaluation and treatment including cytoreductive surgery and hyperthermic intraperitoneal chemotherapy.