Pseudomyxoma peritonei and appendiceal carcinoma with peritoneal metastases: current management strategies

Introduction: Pseudomyxoma peritonei (PMP) is a rare disease that has called worldwide attention to the management of cancer disseminated within the peritoneal surfaces of the abdomen and pelvis. This cancer spread is referred to as peritoneal metastases. In a historical perspective PMP was the first long-term success achieved in the management of peritoneal metastases. Areas covered: The treatment of PMP concerns two essential components. First, the surgeon uses surgical procedures including peritonectomy in order to remove all visible evidence of disease. Following a complete surgical resection, the abdomen is thoroughly washed for an extended time period (60-90 min) with a warm chemotherapy solution. The disease is especially suited for these aggressive local and regional treatment strategies. Expert opinion: The histology of the disease which estimates the aggressive versus benign nature of the process, the preoperative radiology which can estimate small bowel involvement, and the complete visible removal of all tumor constitute the important prognostic indicators by which to estimate the likelihood of long-term survival. A new standard of care for a rare disease has been established which carries a high likelihood of prolonged long-term survival as compared to prior efforts with serial debulking which always ended in a terminal state.