Repeated aggressive surgical debulking results in long-term survival in a patient with urachal adenocarcinoma and pseudomyxoma peritonei

Pseudomyxoma peritonei (PMP) is an unusual condition characterized by massive mucinous ascites associated with peritoneal and omental implantation of tumors. Most cases of PMP arise from the appendix or ovary, but rarely from urachal adenocarcinoma. Although urachal adenocarcinoma with PMP is very difficult to cure and has a poor prognosis, long-term survival can still be achieved with repeated surgical debulking. We report an unusual case of PMP caused by mucinous adenocarcinoma arising from urachus in a 39 year-old man. Direct tumor invasion to the urinary bladder wall, metastasis to the spleen and peritoneal seeding of tumor were found. Surgical debulking combined with intraperitoneal chemotherapy was performed during the first laparotomy inl997. During follow up, recurrent disease was diagnosed and five additional aggressive debulking surgeries were performed. The patient has survived for more than nine years and is doing well.