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Chirurgische Therapie des Pseudomyxoma-peritonei-Syndroms – zytoreduktive Chirurgie und hypertherme intraperitoneale Chemotherapie

Surgical treatment of pseudomyxoma peritonei—Cytoreductive surgery and hyperthermic intraperitoneal chemotherapy

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Zusammenfassung

Hintergrund

Das Pseudomyxoma-peritonei-Syndrom (PMP) ist eine seltene Erkrankung, bei der die Chirurgie die Grundlage der Behandlung darstellt.

Methode

Übersicht zum aktuellen Behandlungsstandard.

Ergebnisse

Zytoreduktive Chirurgie (CRS) in Kombination mit der hyperthermen intraperitonealen Chemotherapie (HIPEC) bilden die Grundlage der Therapie des PMP. Der Peritonealkarzinoseindex sollte präoperativ anhand der Bildgebung und/oder Laparoskopie bestimmt, intraoperativ validiert und jeweils dokumentiert werden. Eine extraperitoneale Präparation ermöglicht eine weitreichende En-block-Resektion des Peritoneums und der befallenen viszeralen Areale. Die HIPEC sollte mit Mitomycin C über 60–90 min erfolgen. Die vollständige CRS (CC = 0, CC = 1) und der histologische Subtyp sind für die Prognose relevant. Strukturierte Ausbildungsprogramme und Mentoring können die Lernkurve optimieren. Die Nachsorge sollte am operativen Zentrum erfolgen. Nach Ausgangsbildgebung 3 Monate nach CRS sollte in den erst 2 Jahren alle 6 Monate eine Kontrolle erfolgen. Danach können die Intervalle auf 1 Jahr verlängert werden.

Schlussfolgerung

Standardisierung chirurgische Therapie und HIPEC, optimierte Ausbildung und strukturierte Nachsorge am Zentrum führen zu einer hervorragender Langzeitprognose des PMP.

Abstract

Background

Pseudomyxoma peritonei syndrome (PMP) is an orphan disease. Surgery is the fundament of treatment.

Method

Short review summarizing the state of the art treatment.

Results

Cytoreductive surgery (CRS) in combination with hyperthermic intraperitoneal chemotherapy (HIPEC) form the foundations of treatment for PMP. The peritoneal cancer index should be preoperatively determined based on imaging and/or laparoscopy, intraoperatively validated and both should be documented. An extraperitoneal surgical preparation technique leads to effective en bloc resection of the peritoneum and the affected abdominal area. The HIPEC technique should be performed with mitomycin C for 60–90 min. Complete CRS (CC = 0, CC = 1) and the histological subtype are relevant for the prognosis. Structured educational programs and mentoring can optimize the learning curve. The aftercare should be performed at the surgical center. After follow-up imaging at 3 months after CRS, in the first 2 years a control should be carried out every 6 months. Thereafter, the intervals can be extended to 1 year.

Conclusion

Standardized surgical treatment and HIPEC, optimized specific surgical training and structured follow-up at the center lead to an excellent long-term prognosis for patients with PMP.

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Literatur

  1. Hinson FL, Ambrose NS (1998) Pseudomyxoma peritonei. Br J Surg 85(10):1332–1339

    Article  CAS  PubMed  Google Scholar 

  2. Smeenk RM, van Velthuysen ML, Verwaal VJ, Zoetmulder FA (2008) Appendiceal neoplasms and pseudomyxoma peritonei: a population based study. Eur J Surg Oncol 34(2):196–201

    Article  CAS  PubMed  Google Scholar 

  3. Lin YL, Xu DZ, Li XB et al (2021) Consensuses and controversies on pseudomyxoma peritonei: a review of the published consensus statements and guidelines. Orphanet J Rare Dis 16(1):85

    Article  PubMed  PubMed Central  Google Scholar 

  4. Sugarbaker PH (2006) New standard of care for appendiceal epithelial neoplasms and pseudomyxoma peritonei syndrome? Lancet Oncol 7(1):69–76

    Article  PubMed  Google Scholar 

  5. Ansari N, Chandrakumaran K, Dayal S, Mohamed F, Cecil TD, Moran BJ (2016) Cytoreductive surgery and hyperthermic intraperitoneal chemotherapy in 1000 patients with perforated appendiceal epithelial tumours. Eur J Surg Oncol 42(7):1035–1041

    Article  CAS  PubMed  Google Scholar 

  6. Jacquet P, Sugarbaker PH (1996) Clinical research methodologies in diagnosis and staging of patients with peritoneal carcinomatosis. Cancer Treat Res 82:359–374

    Article  CAS  PubMed  Google Scholar 

  7. Ihemelandu C (2023) The landmark series: scoring systems for primary peritoneal surface malignancy. Ann Surg Oncol 30(3):1832–1837

    Article  PubMed  Google Scholar 

  8. Yurttas C, Uberruck L, Nadiradze G, Konigsrainer A, Horvath P (2022) Limitations of laparoscopy to assess the peritoneal cancer index and eligibility for cytoreductive surgery with HIPEC in peritoneal metastasis. Langenbecks Arch Surg 407(4):1667–1675

    Article  PubMed  PubMed Central  Google Scholar 

  9. Arjona-Sanchez A, Martinez-Lopez A, Valenzuela-Molina F et al (2022) A proposal for modification of the PSOGI classification according to the Ki-67 proliferation index in pseudomyxoma peritonei. Ann Surg Oncol 29(1):126–136

    Article  PubMed  Google Scholar 

  10. Campos NMF, Almeida V, Curvo Semedo L (2022) Peritoneal disease: key imaging findings that help in the differential diagnosis. Br J Radiol 95(1130):20210346

    Article  PubMed  Google Scholar 

  11. Sugarbaker PH (1995) Peritonectomy procedures. Ann Surg 221(1):29–42

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  12. Chang KH, Kazanowski M, Staunton O et al (2017) Mentored experience of establishing a national peritoneal malignancy programme—Experience of first 50 operative cases. Eur J Surg Oncol 43(2):395–400

    Article  CAS  PubMed  Google Scholar 

  13. Ansari N, Brown KGM, McBride KE et al (2019) Accelerating the learning curve in cytoreductive surgery and hyperthermic intraperitoneal chemotherapy using an external mentor model. ANZ J Surg 89(9):1097–1101

    Article  PubMed  Google Scholar 

  14. Parkin E, Selvasekar C, Wilson M, Renehan A, O’Dwyer S, Aziz O (2019) Laparoscopic cytoreductive surgery with hyperthermic Intraperitoneal chemotherapy (L-CRS/HIPEC) for perforated low-grade appendiceal mucinous neoplasm (LAMN II). Ann Surg Oncol 26(7):2285

    Article  PubMed  Google Scholar 

  15. Wang C, Yin XY, An LB et al (2023) Significance of laparoscopic cytoreductive surgery for appendiceal pseudomyxoma peritonei with limited disease and low tumor burden. Asian J Surg 46(3):1220–1225

    Article  PubMed  Google Scholar 

  16. Mercier F, Jeremie G, Alyami M et al (2020) Long-term results of laparoscopic cytoreductive surgery and HIPEC for the curative treatment of low-grade pseudomyxoma peritonei and multicystic mesothelioma. Surg Endosc 34(11):4916–4923

    Article  PubMed  Google Scholar 

  17. Wen HK, Valle SJ, Morris DL (2023) Bromelain and acetylcysteine (BromAc((R))): a novel approach to the treatment of mucinous tumours. Am J Cancer Res 13(4):1522–1532

    PubMed  PubMed Central  Google Scholar 

  18. Ke K, Pillai K, Mekkawy AH et al (2021) The effect of intraperitoneal administration of BromAc on blood parameters: phase 1 study. Discov Oncol 12(1):25

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  19. Ahmadi N, Kostadinov D, Sakata S et al (2021) Managing recurrent pseudomyxoma peritonei in 430 patients after complete cytoreduction and HIPEC: a dilemma for patients and surgeons. Ann Surg Oncol 28(12):7809–7820

    Article  PubMed  Google Scholar 

  20. Chandrakumaran K, Carr NJ, Mohamed F, Cecil TD, Moran BJ (2023) Development and validation of nomograms to predict survival in patients undergoing complete cytoreduction and hyperthermic intraperitoneal chemotherapy for Pseudomyxoma Peritonei of Appendiceal origin. JAMA Surg 158(5):522–530

    Article  PubMed  Google Scholar 

  21. Govaerts K, Chandrakumaran K, Carr NJ et al (2018) Single centre guidelines for radiological follow-up based on 775 patients treated by cytoreductive surgery and HIPEC for appendiceal pseudomyxoma peritonei. Eur J Surg Oncol 44(9):1371–1377

    Article  PubMed  Google Scholar 

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Authors and Affiliations

  1. Klinik für Allgemein‑, Viszeral‑, Tumor- und Transplantationschirurgie, Uniklinik Köln, Kerpener Straße 62, 50937, Köln, Deutschland

    Roger Wahba FEBS,  Thomas Schmidt,  Denise Buchner,  Tristan Wagner &  Christiane J. Bruns

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  1. Roger Wahba FEBS
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  2. Thomas Schmidt
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  3. Denise Buchner
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  4. Tristan Wagner
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  5. Christiane J. Bruns
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Corresponding author

Correspondence to Roger Wahba FEBS.

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Interessenkonflikt

R. Wahba, T. Schmidt, D. Buchner, T. Wagner und C. Bruns geben an, dass kein Interessenkonflikt besteht.

Für diesen Beitrag wurden von den Autor/-innen keine Studien an Menschen oder Tieren durchgeführt. Für die aufgeführten Studien gelten die jeweils dort angegebenen ethischen Richtlinien.

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C. Bruns, Köln

R. Wahba, Köln

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Wahba, R., Schmidt, T., Buchner, D. et al. Chirurgische Therapie des Pseudomyxoma-peritonei-Syndroms – zytoreduktive Chirurgie und hypertherme intraperitoneale Chemotherapie. Chirurgie 94, 840–844 (2023). https://doi.org/10.1007/s00104-023-01937-3

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