Fact-sheet: Pseudomyxoma peritonei
Updated on 08/26/2020 at 2:09 PM
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Definition
Pseudomyxoma peritonei is a rare entity: 1/1,000,000.
It corresponds to the presence of mucus within the intraperitoneal cavity.
It results from intraperitoneal dissemination of mucus-secreting cells, producing "gelatinous" mucin within the peritoneal cavity.
The main cause is a ruptured mucocele of appendix.
It preferentially affects women between 50 and 60 years of age.
Clinical features
- Asymptomatic for a long time
- Abdominal pain
- Asthenia, anorexia
- Abdominal distension with difficulty tolerating the supine position
- Appearance of subcutaneous nodules
- Appearance of an abdominal wall hernia
Ultrasound
- Echogenic intraperitoneal fluid collection, which may be loculated.
- Small cystic lesions infiltrating around the liver and spleen may be seen = mucinous implants.
- Hepatic and splenic scalloping
- Extrinsic impression on bowel loops.
CT
- Abundant hypodense, loculated ascites with enhancing septations.
- Curvilinear peritoneal calcifications.
- Peritoneal inflammatory reaction: fat infiltration and peritoneal enhancement after contrast injection.
- Hepatic and splenic scalloping.
- Central compartmentalization of bowel loops.
- Peritoneal nodules.
- Fingernail-like calcifications
Search for the primary tumor: Mucocele of appendix, ovarian, colonic, pancreatic cystadenocarcinoma, urachal.
MRI
Reference technique:
- T1: hypointense masses
- T2: hyperintensity due to the fluid content of the cells
- Omental cake
- After gadolinium injection: mild enhancement.
Differentiation of mucinous ascites from solid mucinous lesions.
Management
- Cytoreductive surgery.
- Hyperthermic intraperitoneal chemotherapy
Classification
Peritoneal Cancer Index (PCI) according to Sugarbaker.
Differential diagnosis
- Peritoneal carcinomatosis.
- Sarcoma
- Tuberculous peritonitis.