Chirurgisches Management retroperitonealer Weichteilsarkome – eine Übersicht

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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