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Interdisziplinäre Behandlung der Weichgewebesarkome der Extremitäten

Interdisciplinary treatment of soft tissue sarcoma of the extremities

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Zusammenfassung

Weichgewebesarkome stellen aufgrund ihrer Seltenheit und Heterogenität eine außerordentliche therapeutische Herausforderung dar. Die vollständige chirurgische Entfernung ist und bleibt bei nichtmetastasierten Tumoren die Standardtherapie. Wichtig ist die frühzeitige Einbeziehung interdisziplinärer Therapieansätze durch ein multimodal geschultes Team, um ggf. durch einen chirurgischen Eingriff erst den durch präoperative systemische Chemotherapie und/oder Strahlentherapie devitalisierten Residualtumor zu resezieren. Frühzeitig muss die spätere Funktion der Extremitäten bei der Planung des Eingriffs bedacht werden. Bei lokal fortgeschrittenen T2-Tumoren (> 5 cm), die unter der subkutanen Faszie liegen (T2b) und/oder eine höher maligne Differenzierung (Grading 2 oder 3 nach FNLCC) aufweisen, kann die Operation alleine den Primärtumor i. A. nicht beherrschen. Hier ist eine zusätzliche (neo)adjuvante Therapie sinnvoll und notwendig, und die isolierte Extremitätenperfusion ist heutzutage ein zuverlässiges Standardverfahren. Wichtig ist die frühzeitige Einbeziehung eines in der klinischen Pathologie erfahrenen Pathologen, der nicht nur morphologisch beurteilt, sondern therapierelevante Kenntnisse für Sarkomsubtypen hat. Alveoläre Weichgewebesarkome, solitär fibröse Tumoren, inflammatorische myofibroblastische Tumoren oder das Dermatofibrosarcoma protuberans sind z. B. Weichgewebesarkome, die ähnlich wie das Ewing-Sarkom im Kindes- und Jugendalter durch spezielle molekulare Untersuchungen (FISH) anhand genetischer Translokationen identifizierbar sind und einer spezifischen Therapie bedürfen. Festzulegen sind zusammen mit den anderen Disziplinen die Auswahl, Reihenfolge und Intensität der Therapiemodalitäten. Dies betrifft auch die Radikalität und den Zeitpunkt des chirurgischen Eingriffes selbst. Kenntnisse der Effektivität und Toxizität multimodaler Therapien sind daher für den Chirurgen unabdingbar.

Abstract

Soft tissue sarcomas are a rare and heterogeneous group of tumors. Surgery clearly remains the standard therapy of non-metastatic soft tissue sarcoma. A pretreatment biopsy is necessary to determine the histology and grade of soft tissue sarcomas and to diagnose entities that can be treated by targeted therapies, such as dermatofibrosarcoma protuberans or alveolar soft tissue sarcoma once they are in a metastatic stage. Nevertheless, locally advanced disease requires multimodal treatment and interdisciplinary treatment decisions. Limb sarcoma of borderline resectability (encasement of vessels, invasion of joints or close proximity to motor nerves) may profit from isolated limb perfusion with recombinant tumor necrosis factor and melphalan. Preoperative chemotherapy may be applied in locally advanced high grade tumors when clear resection margins are difficult to achieve. Deep wave hyperthermia has proven to be a useful addition to systemic chemotherapy in such a neoadjuvant setting. Also preoperative radiation therapy has proven to be effective in controlling locally advanced sarcoma despite higher perioperative morbidity which pays off in the long run by better limb function. Postoperative adjuvant external beam irradiation therapy with the best available technique is recommended for any tumor larger than 5 cm with (FNLCC) grades 2 and 3 (American Joint Committee on Cancer stage IIb/III). Given all these therapeutic options, it is absolutely crucial that interdisciplinary decision-making starts early in the therapeutic process. Patients are often seen first by the surgeon. For an optimal treatment surgeons need to know the efficacy and toxicity of the multimodal treatment options described.

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Einhaltung ethischer Richtlinien

Interessenkonflikt. P. Hohenberger: Forschungsförderung und Vertragshonorare von Boehringer Ingelheim; J. Jakob: Vertragshonorare von Boehringer Ingelheim; T. Henzler, B. Kasper und A. Marx geben an, dass kein Interessenkonflikt besteht. Dieser Beitrag beinhaltet keine Studien an Menschen oder Tieren.

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Authors and Affiliations

  1. Chirurgische Klinik, Universitätsmedizin Mannheim, Mannheim, Deutschland

    J. Jakob

  2. Institut für Klinische Radiologie und Nuklearmedizin, Universitätsmedizin Mannheim, Mannheim, Deutschland

    T. Henzler

  3. Sarkomzentrum am Interdisziplinären Tumorzentrum, Universitätsmedizin Mannheim, Mannheim, Deutschland

    B. Kasper

  4. Pathologisches Institut, Universitätsmedizin Mannheim, Mannheim, Deutschland

    A. Marx

  5. Sektion Chirurgische Onkologie, Medizinische Fakultät Mannheim, Chirurgische Klinik, Universität Heidelberg, Universitätsmedizin Mannheim, Theodor-Kutzer-Ufer 1–3, 68167, Mannheim, Deutschland

    P. Hohenberger

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  2. T. Henzler
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  3. B. Kasper
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Correspondence to P. Hohenberger.

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Jakob, J., Henzler, T., Kasper, B. et al. Interdisziplinäre Behandlung der Weichgewebesarkome der Extremitäten. Chirurg 85, 383–390 (2014). https://doi.org/10.1007/s00104-013-2689-3

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