Pseudo-pseudomyxoma peritonei from peritoneal sarcomatosis

Abstract

Background: Pseudomyxoma peritonei (PMP) is a rare clinical entity of mucinous ascites, most commonly associated with appendiceal mucinous neoplasms. Cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) remains the current standard of care for PMP. Peritoneal sarcomatosis (PS) is an exceptionally rare disease with a poor prognosis. PMP associated with PS has not been previously described. The role of cytoreductive surgery and hyperthermic intraperitoneal chemotherapy for PS with or without PMP is not well-defined. PS manifesting like PMP has not been previously described.

Case presentation: A 74-year-old patient with several weeks history of vague abdominal pain and increased abdominal girth was referred to our facility after incidental finding of PMP during laparoscopic inguinal hernia repair. After complete work-up, he was advised to undergo CRS/HIPEC. Intra-operatively, he was noted to have extensive mucinous ascites and underwent aggressive CRS and HIPEC

Result: Final pathology revealed myxoid liposarcoma with associated intraperitoneal mucin dissemination, which was confirmed with cytogenetic analysis.

Conclusion: We describe a rare case of pseudomyxoma peritonei originating from an intra-abdominal sarcoma. We propose the term of “pseudo-pseudomyxoma peritonei” to appropriately describe this unusual clinical event.