Pseudomyxoma peritonei - a report of two cases and a review of the literature

Abstract

Introduction: PMP is a rare disease with a slow but progressive course leading to death. According to the literature PMP encompass wide variety of conditions. To avoid the confusion and to facilitate the treatment and comparison of the results several authors suggested that the term `PMP` should include only the cases with appendiceal origin. Case report: We report two cases with low-grade paseudomyxoma peritonei. The first case was managed by debulking surgery alone with survival 2 years. The second case underwent complete cytoreduction plus HIPEC and 3 years later is still alive and free of disease. There was one patient with a benign appendiceal mucocele treated by appendectomy, who is free of disease 3 years later. Introduction: PMP is a rare disease with a slow but progressive course leading to death. According to the literature PMP encompass wide variety of conditions. To avoid confusion and to facilitate treatment and comparison of the results several authors suggested that the term `PMP` should include only the cases with appendiceal origin. Case report: We report two cases with low-grade paseudomyxoma peritonei. The first case was managed by debulking surgery alone with survival 2 years. The second case underwent complete cytoreduction plus HIPEC and 3 years later is still alive and free of disease. There was one patient with a benign appendiceal mucocele treated by appendectomy, who is free of disease 3 years later. Conclusion: Despite the high complication rate, cytoreductive surgery with HIPEC remains the gold standard in the treatment of pseudomyxoma peritonei. The tumor grade and completeness of cytoreduction are the main prognostic factors. Due to the rarity of the condition most of the surgeons are not experienced enough to manage these patients. Additionally, the incomplete cytoreduction is associated with poorer prognosis and significantly hampers the subsequent interventions. Thus, in the cases when it is found incidentally, the best strategy is the taking of biopsy, appendectomy and subsequent referral to a specialized center for a treatment by multidisciplinary team.