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Aktuelle Behandlungsempfehlungen beim Pseudomyxoma peritonei

Current treatment recommendations for pseudomyxoma peritonei

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Zusammenfassung

Hintergrund

Der Begriff Pseudomyxoma peritonei (PMP) beschreibt ein klinisches Syndrom, gekennzeichnet durch das Vorhandensein von geleeartigen intraperitonealen Schleimansammlungen. Ausgangspunkt ist meist eine Mukozele der Appendix vermiformis. Betroffene Patienten sind lange Zeit asymptomatisch. Die Diagnose wird häufig erst in fortgeschrittenen Stadien gestellt. Das klinische Bild wird durch Tumorausbreitung bestimmt.

Methode

Es erfolgten die Recherche und Auswertung aktueller Literatur.

Ergebnisse

Anhand morphologischer Eigenschaften können PMP-Subtypen mit unterschiedlichem malignem Potenzial differenziert werden. Die Prognose ist abhängig von der histopathologischen Differenzierung und dem klinischen Stadium. Das therapeutische Spektrum reicht von der laparoskopischen Appendektomie bis zur zytoreduktiven Chirurgie („cytoreductive surgery“ [CRS]) und hyperthermen intraperitonealen Chemotherapie (HIPEC).

Schlussfolgerung

Aufgrund der Seltenheit des PMP gibt es keine prospektiv randomisierten Studien. Bezüglich der stadienabhängig günstigsten Therapiestrategie besteht deshalb nicht immer Einigkeit. Die Übersichtsarbeit versucht unter Berücksichtigung des klinischen Bildes und der histologischen Differenzierung therapeutische Optionen für muzinöse Appendixtumoren und das PMP aufzuzeigen.

Abstract

Background

The term pseudomyxoma peritonei (PMP) describes a clinical syndrome characterized by the presence of gelatinous intraperitoneal accumulation of mucus. It mostly originates from a mucocele of the vermiform appendix. Affected patients are often asymptomatic for a long time. Because of its indolent nature it is usually diagnosed at an advanced stage. Clinical presentation is determined by the dissemination of the tumor.

Method

A search and analysis of the current literature were carried out.

Results

Based on the morphological characteristics PMP subtypes with various malignant potential can be differentiated. The prognosis depends on the histopathological differentiation and the clinical stage. The treatment spectrum varies from laparoscopic appendectomy to complete cytoreductive surgery (CRS) with hyperthermic intraperitoneal chemotherapy (HIPEC).

Conclusion

Due to the rarity of PMP there are no prospective randomized studies. Therefore, there is still controversy regarding the best stage-dependent treatment strategy. This review article attempts to clarify the optimal management of mucinous neoplasms of the appendix and PMP taking the clinical presentation and the histological differentiation into consideration.

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  1. Krankenhaus Barmherzige Brüder Regensburg, Prüfeninger Str. 86, 93049, Regensburg, Deutschland

    H. Leebmann & P. Piso

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  1. H. Leebmann
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Correspondence to H. Leebmann.

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H. Leebmann und P. Piso 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

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Leebmann, H., Piso, P. Aktuelle Behandlungsempfehlungen beim Pseudomyxoma peritonei. Chirurgie 93, 1152–1157 (2022). https://doi.org/10.1007/s00104-022-01696-7

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