Tailgut Cyst, an Unusual Cause of Pseudomyxoma Peritonei

Abstract

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.