Tailgut cyst, an unusual cause of pseudomyxoma peritonei.

BACKGROUND Retrorectal hamartoma, also known as tailgut cyst, is a rare tumor that arises from remnants of the embryonic postanal gut. The tumor occurs in the retrorectal space and may undergo malignant degeneration. METHODS The clinical information on a single patient with pseudomyxoma peritonei associated with a tailgut cyst was reviewed and a literature review of the subject performed. RESULTS In this patient there was malignant degeneration of a tailgut cyst localized to the pelvis. Twenty months later a local recurrence in the pelvis and pseudomyxoma peritonei widely distributed in the abdomen were successfully treated by cytoreductive surgery and perioperative chemotherapy. CONCLUSION; Tailgut cyst may, if resected with tumor spillage, result in pseudomyxoma peritonei. Our treatment--which has maintained a disease-free status for one year--was cytoreductive surgery plus perioperative chemotherapy.