Pseudomyxoma peritonei

Caused by cystoadenocarcinoma of the appendix or colon with seeding of mucin producing cells to the peritoneum.

Mucinous appendiceal neoplasms are the leading cause.

A consequence of a ruptured mutinous cystadenoma or cystadenocarcinoma of the appendix or ovaries.

The majority of pseudomyxoma peritoneal,the diffuse collection of gelatinous material in the abdomen and pelvis, and mucinous implants of the peritoneal surfaces, arise from the appendix and represent local spread into the peritoneal cavity.

It is a clinical condition characterized by progressive accumulation of mucineous ascities and peritoneal implants with fatal outcome.

Generally discovered intraoperatively, with an incidence of 2 in every 10,000 laparotomies.

Characterized by accumulation of gelatinous fluid and implants within the abdominal cavity.

Female predominance.

Mucin migrates diffusely throughout the abdominal cavity, seeds the peritoneal surfaces of the viscera, abdominal wall and peritoneum.

Most commonly related to appendiceal primary.

Treatment consists of surgical debulking and intraperitoneal chemotherapy.

Tumor of low biological aggressiveness and not associated with lymph node or liver metastases.

Minimal invasiveness and extensive layering on the peritoneal surfaces.

Patients with complete surgical cytoreduction and intraperitoneal chemotherapy have a 5 year survival of 86%, while patients with incomplete cytoreduction have a 20% 5 year survival.

Treatment include cytoreduction surgery with hyperthermic intraperitoneal chemoperfusion.
Systemic chemotherapy is contraindicated.
Recurrences typically managed by  repeat resection and hyperthermic intraperitoneal chemo perfusion.
Perioperative morbidity with intra-abdominal abscess, anastmotic leakage and wound complications are common  at 8-41%. 
Mortality is rare.

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