Mucocele and Pseudomyxoma Peritonei

Posted by e-Medical PPT Tuesday, August 9, 2011
Extremely rare; 300 cases/yr in U.S.
Diffuse, intraperitoneal collection of gelatinous fluid with mucinous tumor implants on peritoneal surfaces and omentum
Strictly, etiology is 2nd grade I mucinous cystadenocarcinoma of the appendix
Ovarian, pancreatic cancer will have similar picture

Pathophysiology
Mucocele rupture  dissemination of mucin-producing tumor cells throughout peritoneal cavity
Characteristic and predictable pattern of tumor progression:
Gravity  dependent collection of tumor
    (pelvis, retrohepatic space, paracolic gutters, Treitz)
2) Resorption of peritoneal fluid  accumulation of tumor cells to distinct sites:

Deposit Sites 2nd Fluid Resorption
1) Between liver, R hemidiaphragm
        -2nd  lymphatics within undersurface of hemidiaphragm
2) Greater, lesser omentum
        -lymphatics draw fluid, attracting tumor cells to their surface  omental caking

Sugarbaker Protocol
Radical debulking of tumor load:
appendix, peritoneum, omentum;
additional viscera as indicated
Curative therapy = all nodules > 2.5 mm

Intraoperative heated mitomycin
Post-operative 5-FU
Reports of 80% 10 yr survival

Rationale for Radical Surgery
Low aggressiveness of tumor; rare LN or liver involvement
Peritoneal dissemination occurs early
Areas of spread are treatable by peritonectomy/omentectomy
Redistribution phenomenon: small bowel is largely spared (2nd motility?)
Regional chemotherapy can attack all surfaces exposed to tumor

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