The influence of tumor cell entrapment phenomenon on the natural history of Pseudomyxoma peritonei syndrome.

Abstract

Pseudomyxoma peritonei syndrome (PMP) may be associated with slow "benign" or malignant process. However, the natural history of this disease is slow progression to death. Its treatment is variable and controversial. In this current study we have compared the patients with Pseudomyxoma peritonei syndrome with recurrences due to the cell entrapment hypothesis. Data were derived from a database of patients with PMP treated at our hospitals from 2004 to 2009. All patients had undergone various surgical operations prior to referral to our institutions for definitive treatment. All patients had recurrences in special sites due to entrapment of malignant cells. There are 6 patients, four men and two women. The initial clinical presentation of the disease was hernia in one, appendicitis in three and ovarian mass in one. The mean time from the initial operation to be definitive management was 23.5 months with an average of 1.83 operations per patient. After cytoreduction and hyperthermic intraperitoneal chemotherapy (HIPEC) the mean survival was 31 months with minimal recurrences and only 0.3 operations per patient. Our data suggest that the patients should be referred to a center with a peritoneal surface malignancy program after the PMP diagnosis as soon as possible. Incomplete debulking procedures and minimal invasive operations promote uncontrollable intraabdominal tumor growth due to tumor cell entrapment and the tendency of PMP to grow at wound sites.