Pseudomyxoma Peritonei

Introduction

• Description: Pseudomyxoma peritonei is the intraperitoneal spread of a mucin-secreting tumor (either a mucinous cystadenoma or carcinoma), which results in recurrent abdominal masses, often-massive ascites, and multiple bowel obstructions. This tumor most frequently begins in the appendix.

• Prevalence: Roughly 3500 cases per year (male and female). 2/10,000 laparotomies and 2%–5% of ovarian mucinous tumors (16% in mucinous cystadenocarcinomas).

• Predominant Age: Middle to late reproductive age and early postmenopausal years.

• Genetics: No genetic pattern.

Etiology and Pathogenesis

• Causes: Spread, rupture, spill, or leakage of a primary appendiceal tumor or other gastrointestinal or ovarian tumor. Recent histologic studies suggest that in the majority of patients the appendix is the primary tumor source. In rare cases, metaplasia by the cells of the peritoneal surface may account for this tumor.

• Risk Factors: Rupture or leakage of an ovarian mucinous tumor at the time of surgical resection. This role has been debated in recent literature.

Signs and Symptoms

• Accumulation of large amounts of mucinous material in the peritoneal cavity

• Recurrent bowel obstruction

• Implants of tumor on the omentum, undersurface of the diaphragm, pelvis, right retrohepatic space, left abdominal gutter, and ligament of Treitz. (In contrast to carcinoma the peritoneal surface of the bowel is generally spared; metastasis outside the peritoneal cavity does not occur.)

Diagnostic Approach