Multimodale Therapie primärer, nicht metastasierter retroperitonealer Sarkome

Zusammenfassung

Weichteilsarkome sind eine heterogene Gruppe von Tumoren mesenchymalen Ursprungs, die teilweise ein sehr unterschiedliches biologisches Verhalten zeigen. Retroperitoneale Weichteilsarkome (RSTS) machen 10 – 15% aller Weichteilsarkome aus. Behandlungsempfehlungen für RSTS basieren auf vorrangig retrospektiven Analysen mit geringer Evidenz, die in dieser Übersichtsarbeit dargestellt werden. Zentraler Pfeiler in der Behandlung des lokalisierten, nicht metastasierten RSTS ist die chirurgische Resektion im Sinne einer Kompartment-Resektion. Ziel ist dabei immer die allseitig vollständige Tumorentfernung mit mikroskopisch freien Resektionsrändern. Ist das Erreichen dieses Behandlungsziels mit der alleinigen Kompartment-Resektion fraglich oder besteht eine hohe Wahrscheinlichkeit für eine lokale Tumorrekurrenz bei high-grade Sarkomen, so sollte eine neoadjuvante Bestrahlung, gegebenenfalls mit intraoperativem Boost, im Rahmen eines multimodalen Therapieansatzes durchgeführt werden. Die in Analogie zu den Extremitätensarkomen zu favorisierende Anthrazyklin-basierte Chemotherapie hat ihren Stellenwert vorrangig in der Adjuvanz. Auch wenn ein onkologischer Vorteil durch eine adjuvante Chemotherapie für RSTS bisher nicht nachgewiesen ist, sollte bei high-grade Sarkomen die Indikation diskutiert werden. In Kenntnis der unterschiedlichen Krankheitsverläufe werden zunehmend an die unterschiedlichen histologischen Subtypen adaptierte Therapiekonzepte diskutiert und in Studien untersucht. Valide Empfehlungen lassen sich jedoch bisher nicht ableiten. Die Behandlung von RSTS-Patienten wie überhaupt von Patienten mit Sarkomen in Zentren mit entsprechender Expertise ist mit einer signifikanten Verbesserung der Prognose assoziiert und ist unbedingt anzustreben.

Schlüsselwörter

retroperitoneales Sarkom - multimodale Therapie - Kompartmentresektion - Bestrahlung - Chemotherapie

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