Pseudomyxoma Peritonei

Česká verzia

Authors: Petr Bartoška ¹; František Antoš ¹; Pavel Vítek ²; Josef Marx ¹; Jiří Kopic ¹; Petra Holečková ³
Authors place of work: Chirurgická klinika 1. LF UK a Nemocnice Na Bulovce, Praha ¹; Protonové centrum, Praha ²; Ústav radiační onkologie, 1. LF UK a Nemocnice Na Bulovce, Praha ³
Published in the journal: Klin Onkol 2019; 32(5): 329-332
Category: Review
doi: https://doi.org/10.14735/amko2019329

Summary

Background: Pseudomyxoma peritonei is a rare tumorous disease with various grades of malignancy and is characterized by production of mucinous and gelatinous masses. Development of pseudomyxoma peritonei is usually associated with rupture of appendiceal mucinous tumors and other mucinous tumors of the gastrointestinal tract or ovaries. Pseudomyxoma peritonei is usually divided into three types: low-grade, high-grade, and high-grade with signet ring cells. Staging of the disease is determined by the peritoneal cancer index. Clinical findings are highly variable depending on disease staging. The typical finding of “jelly belly” syndrome worsens with disease progression. The diagnosis is based on the pre-operative cure by imaging methods, especially computed tomography.

Methods: The Sugarbaker method involves maximal removal of tumorous masses, so-called cytoreductive surgery, and perioperative intraabdominal application of hot cytostatics (hyperthermic intraperitoneal chemotherapy) with the aim of achieving maximal liquidation of tumorous processes.

Results: Our results are comparable with previous published data and confirm high effectivness of this method. The results show statistically very significant extention of overall survival, disease free interval with acceptable lethality 0–12 % and morbidity 27–56%. These results promote this method as the gold standard of treatment of pseudomyxoma peritonei in selected patients.

Keywords:

cytoreductive surgery – pseudomyxoma peritonei – PCI – hypertermic intraperitoneal chemotherapy – HIPEC

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