Clinical presentation of pseudomyxoma peritonei

Abstract

Background and aims: We analysed the clinical symptoms and signs of pseudomyxoma peritonei (PMP), a rare syndrome affecting one to two persons per million annually. Presumably, patients with PMP would benefit from early diagnosis. This study was conducted to further characterise the manifestations of PMP.

Material and methods: We performed a retrospective chart review of a consecutive series of 82 PMP cases at the Helsinki University Central Hospital from June 1984 to September 2009. We then analysed the patients' characteristics, clinical manifestations, indications for surgery and preoperative radiological investigations.

Results: The study involved 53 women (65%) and 29 men (35%). The preponderance of women was statistically significant (p = 0.008). Abdominal pain was the most common chief complaint in the initial evaluation with 23% of the cases. The subsequent more common chief complaints were acute abdomen with 21%, increased abdominal girth with 17%, coincidental diagnosis with 13%, other reasons with 13% and newly onset hernia with 12% of the cases. Suspected ovarian tumour was the most common cause for surgery overall, with 26 of 82 initial operations (32%). Of the 82 initial operations, 23 (28%) were performed with a suspicion or diagnosis of PMP. Computed tomography had a sensitivity of 51% in recognising PMP.

Conclusions: While only 28% of patients underwent initial surgery for presumed PMP, the accuracy of preoperative diagnostics was modest. Diagnostics of PMP is challenging due to the mimicking nature of the disease. In case of indistinct intra-abdominal tumour, we recommend, careful patient examination prior to the initial surgery, when possible.