Pseudomyxoma peritonei

Abstract

Pseudomyxoma peritonei (PMP) is a rare condition, which is known for its high mortality when not treated properly. The first step to improve prognosis of these patients is to recognize this clinical syndrome preferably in an early stage. Knowledge of pathogenesis and common diagnostic tools is essential in this regard. Treatment strategy for PMP should pursue complete cytoreduction and prevention of recurrence or progression. Combined modality treatment, consisting of cytoreductive surgery with hyperthermic intraperitoneal chemotherapy, seems very efficient in this regard. This approach is currently carried out in many centers throughout the world with promising results and seems to win ground as the standard treatment approach.

Mucinous Adenocarcinomas with Intra-Abdominal Dissemination: A Review of Current Therapy

“…Favorable results with combined modality treatment have been achieved for patients with benign disease (DPAM) and complete cytoreduction, with 5-and 10-year survival rates of approximately 75%-100% and 68%, respectively [9,10,41]. However, patients with malignant disease (PMCA) or intermediate disease (PMCA-I) showed a significantly worse prognosis, with 5-and 10-year survival rates, respectively, of 50% and 21% for PMCA-I and 14% and 3% for PMCA [9].…”

“…It is a locoregional disease within the abdomen, characterized by mucinous tumor on peritoneal surfaces producing excessive amounts of mucinous ascites [10]. The mucinous feature in PMP is mucinous ascites and not intracellular mucin accumulation in tumor cells.…”

“…Accumulation and the reproduction of free and implanted tumor cells leads to progressive peritoneal mucinous tumor and ascites, but invasion of the peritoneal surface usually remains absent. The locoregional progression-also referred to as redistribution phenomenon [14]-of the cancer results in a starvation phase of the disease and death resulting from the failure of intestinal function because of intraabdominal pressure, fistula formation, or infection [10]. In the past it was a uniformly lethal condition.…”

Rare cases of appendicitis presented in KIMS Amalapuram, India: a clinical study

“…However, conclusions cannot be drawn from ultra-sonography alone since the mucenous ascitis resembles free peritoneal fluid. 10 Computed tomography shows higher densities of collections (mucinous ascites) compared to nonmucinous collections, which is characteristic pattern of mucinous accumulation and the extent of disease for preoperative planning and prognostic purposes.…”

Pseudomyxoma extraperitonei of ovarian origin mimicking a psoas abscess

“…Sugarbaker reported curative treatment of malignant PMP with aggressive cyto-reductive surgery and adjuvant intraperitoneal chemotherapy [10]. Surgical de-bulking is the standard treatment, but combined modality treatment, consisting of aggressive peritonectomy with intra-operative hyperthemic intra-peritoneal chemotherapy is the new recommended standard approach [11].…”