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Neue diagnostische und therapeutische Aspekte zu Chordomen

Novel diagnostic and therapeutic aspects on chordomas

  • Leitthema
  • Published:
Der Onkologe Aims and scope

Zusammenfassung

Hintergrund

Chordome zählen zu den primären, malignen Knochentumoren und treten mit Schwerpunkt im Sakrum und Clivus entlang der Wirbelsäule auf (ICD-O: 9370/3). Man geht davon aus, dass sie aus Resten der Chorda dorsalis entstehen. Aufgrund des seltenen Auftretens zählen sie zu den sog. „orphan diseases“.

Methoden

Es wurden eine Recherche und Auswertung aktueller Literatur und klinischer Studien durchgeführt.

Ergebnisse

Die primäre Therapie stellt heutzutage die Chirurgie dar; allerdings kann dies v. a. im Clivus problematisch sein. Zudem treten häufig Rezidive und nicht selten Metastasen auf. In der rezidivierten Situation wird die Hochdosisbestrahlung mit Protonen empfohlen. Aufgrund des generell langsamen Wachstums der Chordome ist eine konventionelle Chemotherapie problematisch. In den letzten Jahren konnten für Chordome molekularpathologische Signalwege mit entsprechenden Zielstrukturen identifiziert werden, die erste Ansätze für eine zielgerichtete Therapie bieten.

Abstract

Background

Chordomas are rare, primarily malignant bone tumors with a particular high incidence at the sacrum and the clivus along the spine (ICD-O: 9370/39). Chordomas are assumed to arise from remnants of the chorda dorsalis. Due to the rarity of chordomas they belong to the group of so-called orphan diseases.

Methods

A search and evaluation of the current literature and clinical studies were carried out.

Results

The first choice of therapy is a complete surgical resection, which can be difficult particularly in the clivus region. In addition, recurrence often occurs and metastases are not uncommon. In the recurrency situation high-dose proton radiation is recommended. Due to the generally slow growth of chordomas conventional chemotherapy is largely ineffective. In recent years molecular genetic pathways with the corresponding target structures have been identified in chordomas, which offer the first approach for a targeted therapy.

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Authors and Affiliations

  1. Institut für Pathologie, Universitätsklinik Ulm, Albert-Einstein-Allee 23, 89081, Ulm, Deutschland

    K. Mellert, P. Möller &  T. F. E. Barth

  2. Kopf-Hals-Tumorzentrum Ulm, Klinik für Hals-Nasen-Ohrenheilkunde und Kopf-Hals-Chirurgie, Universitätsklinik Ulm, Frauensteige 12, 89070, Ulm, Deutschland

    A. von Witzleben

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  1. A. von Witzleben
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Correspondence to T. F. E. Barth.

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Interessenkonflikt

A. von Witzleben, K. Mellert, P. Möller und T.F.E. Barth geben an, dass kein Interessenkonflikt besteht.

Dieser Beitrag beinhaltet keine von den Autoren durchgeführten Studien an Menschen oder Tieren.

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von Witzleben, A., Mellert, K., Möller, P. et al. Neue diagnostische und therapeutische Aspekte zu Chordomen. Onkologe 24, 199–207 (2018). https://doi.org/10.1007/s00761-017-0327-2

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