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World J Gastrointest Oncol. Jan 15, 2010; 2(1): 44-50
Published online Jan 15, 2010. doi: 10.4251/wjgo.v2.i1.44
Pseudomyxoma peritonei
Katharine E Bevan, Faheez Mohamed, Brendan J Moran
Katharine E Bevan, Faheez Mohamed, Brendan J Moran, Pseudomyxoma Peritonei Centre, Basingstoke and North Hampshire Hospital Foundation Trust, Aldermaston Road, Basingstoke, Hampshire RG24 9NA, United Kingdom
Author contributions: Bevan KE, Mohamed F and Moran BJ reviewed the literature and wrote the manuscript; Moran BJ edited the final draft.
Correspondence to: Brendan J Moran, MCh, FRCS, Pseudomyxoma Peritonei Centre, Basingstoke and North Hampshire Hospital Foundation Trust, Aldermaston Road, Basingstoke, Hampshire RG24 9NA, United Kingdom. brendan.moran@bnhft.nhs.uk
Telephone: +44-1256-314709 Fax: +44-1256-313512
Received: July 2, 2009
Revised: December 24, 2009
Accepted: December 31, 2009
Published online: January 15, 2010
Abstract

Pseudomyxoma peritonei (PMP) is an uncommon “borderline malignancy” generally arising from a perforated appendiceal epithelial tumour. Optimal treatment involves a combination of cytoreductive surgery (CRS) with heated intraperitoneal chemotherapy (HIPEC). Controversy persists regarding the pathological classification and its prognostic value. Computed tomography scanning is the optimal preoperative staging technique. Tumour marker elevations correlate with worse prognosis and increased recurrence rates. Following CRS with HIPEC, 5-year survival ranges from 62.5% to 100% for low grade, and 0%-65% for high grade disease. Treatment related morbidity and mortality ranges from 12 to 67.6%, and 0 to 9%, respectively. Surgery and HIPEC are the optimal treatment for PMP which is at best a “borderline” peritoneal malignancy.

Keywords: Pseudomyxoma peritonei, Cytoreductive surgery, Heated intraperitoneal chemotherapy, Jelly belly, Appendiceal mucinous tumour, Peritoneal malignancy, Borderline malignancy