Zusammenfassung
Einleitung: Retroperitoneale Weichteilsarkome sind seltene und sehr heterogene Tumoren, deren
therapeutisches Management nur teilweise standardisiert ist. Ziel der vorliegenden
Übersichtsarbeit ist, anhand einer selektiven Recherche der neueren Literatur den
aktuellen Stand der Klassifikation, Diagnostik und chirurgischen/multimodalen Therapie
der retroperitonealen Weichteilsarkome zusammenzufassen. Methode: Klinisch basierter empirischer narrativer Review mit besonderer Berücksichtigung
der Literatur der letzten 5 Jahre. Ergebnisse: Aufgrund des Fehlens prospektiv-randomisierter Studien orientiert sich die Therapie
retroperitonealer Weichteilsarkome in weiten Teilen an persönlicher Erfahrung, retrospektiv
erhobenen Daten und dem Vergleich mit historischen Kontrollen. Die Diagnostik schließt
eine genaue morphologische Abgrenzung des Tumors durch Schnittbildverfahren (CT, MRT)
sowie in aller Regel die histologische Sicherung durch geschlossene oder offene Biopsie
ein. Die entscheidende Säule der Therapie ist die komplette chirurgische Tumorentfernung,
wobei sich die prognostische Bedeutung des mikroskopisch tumorfreien Resektionsrandes
in neueren Studien bestätigen ließ und deshalb in den letzten Jahren ein Trend zu
zunehmend radikaleren Resektionen erkennbar ist. Die Chemotherapie spielt in der adjuvanten
Situation derzeit keine Rolle außerhalb von Studien. Die Rolle der adjuvanten Strahlentherapie
wird kontrovers diskutiert; durch neue Bestrahlungstechniken und die verstärkte präoperative
Anwendung der Bestrahlung wird versucht, dem Problem der behandlungsassoziierten Toxizität
zu begegnen. Auch beim Lokalrezidiv sowie beim Vorliegen von Lungenmetastasen stellt
die Resektion, wann immer möglich, die Therapie der Wahl dar. Neben der Qualität der
chirurgischen Therapie spielen krankheitsspezifische Faktoren (histologischer Tumortyp,
Differenzierungsgrad) eine Rolle für die Prognose. Schlussfolgerung: Das Management retroperitonealer Weichteilsarkome ist komplex und in weiten Teilen
nicht evidenzbasiert. Aufgrund der Seltenheit der Tumoren, der benötigten Erfahrung
und der Vielzahl der beteiligten Fachdisziplinen sollten Diagnostik, Behandlung und
Nachsorge generell in einem Zentrum erfolgen. Zum Erreichen einer R0-Situation sind
nach Risiko-Nutzen-Abwägung auch multiviszerale Resektionen indiziert. Die Entscheidung
zur additiven Strahlentherapie muss individualisiert unter Berücksichtung patienteneigener
und tumorspezifischer Faktoren sowie des Resektionsstatus getroffen werden.
Abstract
Introduction: Retroperitoneal soft-tissue sarcomas (RSTS) represent a rare and heterogeneous class
of diseases for which the clinical management is still largely non-standardised. Based
on a selective review of recent publications, it was the purpose of the present review
article to summarize the current concepts of disease classification, diagnostics and
surgical as well as multimodal therapy for these tumors. Method: A clinically based empirical review derived from a literature search focusing on
publications from the past 5 years was carried out. Results: Due to the paucity of randomised-controlled trials, therapy for RSTS is largely based
on personal experience, retrospectively gathered data and historical controls. Pre-therapeutic
planning requires precise information on the localisation, extension, and texture
of the tumor through cross-sectional imaging (CT, MRI) as well as histological diagnosis
through percutaneous or open biopsy. Complete tumor resection is crucial. Recent studies
have confirmed the importance of microscopically negative resection margins which
has subsequently led to a trend towards more radical resection. Chemotherapy does
not play a role in the adjuvant setting except in clinical trials; however, radiotherapy
has been controversely debated in adjuvant RSTS therapy. Efforts to limit radiation
toxicity include modern techniques as well as a strategy of using pre-resection radiotherapy
instead of postoperative radiation. Surgery is also the treatment of choice for locally
recurrent RSTS and pulmonary metastases. The prognosis of RSTS depends on the quality
of surgical care and several disease-specific factors (histological type, grading).
Conclusion: The clinical management of RSTS is complex and can only partly be considered as evidence-based.
Due to the required level of experience in the treatment of these tumor lesions and
the involvement of several subspecialties, pre-therapeutic planning, treatment and
follow-up should be limited to high-volume surgical centres. In order to achieve microscopically
negative resection margins, multivisceral resections are a valuable option after thorough
consideration of the risks and benefits. Adjuvant radiotherapy needs to be decided
upon on an individual basis, taking into account patient- and tumor-specific factors
as well as resection status.
Schlüsselwörter
retroperitoneales Weichteilsarkom - Literaturübersicht - chirurgisches Management
- multimodale Therapie
Key words
retroperitoneal soft-tissue sarcoma - review of literature - surgical management -
multimodal therapy
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Dr. B. Garlipp
Universitätsklinikum Magdeburg A. ö. R. · Klinik für Allgemein-, Viszeral- und Gefäßchirurgie
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Deutschland
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