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Pseudomyxoma peritonei (PMP) and its therapy – 20 years’ experience of a single surgical department

Česká verzia

Authors: P. Bartoška 1;  F. Antoš 1;  P. Vítek 2;  J. Marx 1;  P. Holečková 3;  M. Novotný 1;  M. Kengbaeva 1
Authors place of work: Chirurgická klinika 1. lékařské fakulty Univerzity Karlovy a Nemocnice Na Bulovce, Praha, subkatedra koloproktologie IPVZ Praha 1;  Proton Therapy Center Czech, Praha 2;  Ústav radiační onkologie, Nemocnice Na Bulovce a 1. lékařské fakulty Univerzity Karlovy, Praha 3
Published in the journal: Rozhl. Chir., 2020, roč. 99, č. 4, s. 159-166.
Category: Original articles
doi: https://doi.org/10.33699/PIS.2020.99.4.159–166

Summary

Introduction: Pseudomyxoma peritonei (PMP) is a rare malignant disease with various grades of malignancy, producing mucinous and gelatinous masses. The origin of PMP is usually connected with the rupture of appendiceal mucinous tumours, other mucinous tumours of the gastrointestinal tract or of the ovary. The staging of this disease is determined by the PCI score (peritoneal cancer index), and the efficiency of surgical procedure by the CC score. Clinical presentation is very variable and depends on the stage of the disease. Many patients are asymptomatic with a minimal clinical finding, presented only with abdominal discomfort. A typical finding of the “jelly belly“ syndrome expands with progression of the disease. The diagnosis consists in preoperative determination of the tumour characteristics and PCI based on imaging methods, especially CT imaging.

Methods: The Sugarbaker technique of complete tumour removal or the so-called cytoreductive surgery (CRS) was used, including hyperthermic intraperitoneal chemotherapy (HIPEC) or alternatively early postoperative intraperitoneal chemotherapy (EPIC). We performed retrospective evalu­ation of 73 patients with pseudomyxoma peritonei undergoing surgery, 39 males and 34 females, mean age 50.6 and 56.4 years, respectively. Surgical revision was performed in 18 patients, 14 males and 4 females. The mean age of this group was 48.8 for the males and 47 for the females. The surgical procedures were performed between 1999 and 2018. Survival rates, median survival, complications based on Clavien-Dindo classification, lethality rates, and PCI and CC scores were assessed in the patient group.

Results: 96 surgeries were performed in 73 patients with pseudomyxoma peritonei at our surgical department between 1999−2018. The surgery had to be repeated in 18 patients (24.6%). High grade (HG) pseudomyxoma was diagnosed in 29 patients (39.7%), and low grade (LG) pseudomyxoma in 44 patients (60.3%). Overall morbidity was 27.3%, and the mortality rate was 5.4%. The mean overall survival (OS) was 139.5 months in the LG pseudomyxoma group and 71.5 months in the HG pseudomyxoma group. Median survival was 86 months in the entire group and 72 in the HG pseudomyxoma group; the median was not reached in the LG pseudomyxoma group.

Conclusions: Results in the literature and our results are comparable, confirming the high efficiency of this method both in the world and in the Czech republic. The results indicate a highly statistically significant improvement of the OS with acceptable mortality and morbidity. These results confirm this method as a gold standard therapy for selected patients.

Keywords:

pseudomyxoma peritonei – HIPEC – hyperthermic intraperitoneal chemotherapy – peritoneal carcinoma index – cytoreductive surgery

Zdroje
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