Controversy and consensus on the management of patients with pseudomyxoma peritonei.

Pseudomyxoma peritonei and peritoneal carcinomatosis derived from appendiceal epithelial tumors are controversial entities from classification to treatment. For the former entity, multiple classification systems have been attempted to distinguish indolent from more aggressive subtypes. The treatment of the low grade variants is managed with serial cytoreduction surgery, with data indicating possible, but unproven, benefit from heated intraperitoneal chemotherapy (HIPEC). There is no consensus on the role of cytoreduction and HIPEC for the management of the more aggressive histologic variants and peritoneal carcinomatosis. Currently, we believe systemic chemotherapy is the standard of care for patients with the high grade variants and peritoneal carcinomatosis.