Pseudomyxoma peritonei

Abstract

Pseudomyxoma peritonei (PMP) is an uncommon borderline malignancy simulating ovarian cancer clinically, radiologically and at surgery. Whilst most cases are now considered to originate from a perforated mucinous appendiceal tumour, ovarian masses are one of the commonest clinical presenting features of PMP in women. The clinical presentation of jelly belly represents mucinous ascites production from abnormal intraperitoneal cells. Controversy persists regarding pathological classification for PMP. CT scanning is the optimal preoperative staging technique. Tumour marker elevations correlate with worse prognosis and increased recurrence rates. Optimal treatment involves a combination of cytoreductive surgery (CRS) with heated intraperitoneal chemotherapy (HIPEC). The detection of mucin or jelly in the abdominal cavity should always point towards the possibility of an appendiceal tumour and careful assessment with appendicectomy, if feasible, is recommended. © 2011 Springer-Verlag Berlin Heidelberg.