Journal article Open Access
Bobovec, Dino; Jurlin, Lana; Majerovic, Mate
Background: Pseudomyxoma peritonei is a rare clinical condition characterized by mucin-secreting epithelial cells which lead to formation of jelly-like structures within the peritoneal cavity and the accumulation of mucinous ascites. Most commonly it arises from the intra-abdominal spread of appendiceal mucinous tumors. Few therapeutic options exist, but a combination of cytoreductive surgery and hyperthermic intraperitoneal chemotherapy, as described by Sugarbaker, stands for the treatment of choice in many tertiary centers nowadays.
Case study: We present a 62-year-old female patient who was initially presented as acute appendicitis. Later pathohistological diagnosis of Pseudomyxoma peritonei just confirmed intraoperative suspicions. Definitive diagnosis was followed with a complete cytoreductive surgery and hyperthermic intraperitoneal chemotherapy. After a 10 year relapse-free follow up, she presented with colon adenocarcinoma and a recurrence of jelly-like incapsulated structures within the abdominal cavity.
Conclusion: In patients diagnosed with pseudomyxoma peritonei, according to current findings, best results are achieved using complete cytoreductive surgery and hyperthermic intraperitoneal chemotherapy. However, recurrences still do occur and there is no real consensus regarding their optimal treatment.
ACC2019No1 Relapse of the pseudomyxoma peritonei after a cytoreductive surgery with peritonectomy and hipec.pdf
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