BACKGROUND/AIMS: Pseudomyxoma peritonei is a rare disease characterized by diffuse intraperitoneal mucinous tumor and massive mucinous ascites. The mainstay of treatment is surgery in combination with adjuvant therapy. METHODOLOGY: From 1995 to 2002, 8 patients with pathologically confirmed pseudomyxoma peritonei were studied. After surgical debulking, intraoperative intraperitoneal hyperthermia was performed followed by 5 days of postoperative intraperitoneal hyperthermia. Normal saline at 46 C was used for hyperthermia. Patients were followed for clinical data, survival, morbidity, and mortality. RESULTS: The appendix was the origin of pseudomyxoma peritonei in 6 patients, the ovary in 1 patient, and the fallopian tube in another. The pathologic diagnosis was mucinous adenocarcinoma in 4 patients, mucinous cystadenocarcinoma in 3, and mucinous cystadenoma in 1. Associated morbidity and mortality was 12.5% and 0, respectively. The recurrence rate was 62.5%, with a median follow-up of 52.5 months. The estimated 1-, 3-, and 5-year survival rates were 100%, 88%, and 49%, respectively. CONCLUSIONS: Surgical debulking followed by 46 C normal saline intraperitoneal hyperthermia resulted in a 5-year survival of 49% with low morbidity and no mortality for the treatment of pseudomyxoma peritonei. We concluded that this is an easy, safe, and efficacious treatment for patients with this rare disease.
BACKGROUND/AIMS: Pseudomyxoma peritonei is a rare disease characterized by diffuse intraperitoneal mucinous tumor and massive mucinous ascites. The mainstay of treatment is surgery in combination with adjuvant therapy. METHODOLOGY: From 1995 to 2002, 8 patients with pathologically confirmed pseudomyxoma peritonei were studied. After surgical debulking, intraoperative intraperitoneal hyperthermia was performed followed by 5 days of postoperative intraperitoneal hyperthermia. Normal saline at 46 C was used for hyperthermia. Patients were followed for clinical data, survival, morbidity, and mortality. RESULTS: The appendix was the origin of pseudomyxoma peritonei in 6 patients, the ovary in 1 patient, and the fallopian tube in another. The pathologic diagnosis was mucinous adenocarcinoma in 4 patients, mucinous cystadenocarcinoma in 3, and mucinous cystadenoma in 1. Associated morbidity and mortality was 12.5% and 0, respectively. The recurrence rate was 62.5%, with a median follow-up of 52.5 months. The estimated 1-, 3-, and 5-year survival rates were 100%, 88%, and 49%, respectively. CONCLUSIONS: Surgical debulking followed by 46 C normal saline intraperitoneal hyperthermia resulted in a 5-year survival of 49% with low morbidity and no mortality for the treatment of pseudomyxoma peritonei. We concluded that this is an easy, safe, and efficacious treatment for patients with this rare disease.