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Notochordale Tumoren

Benigne notochordale Tumoren und Chordome

Notochordal tumors

Benign notochordal tumors and chordomas

  • Schwerpunkt: Knorpel, Knochen, Chorda – Molekulare Pathologie
  • Published:
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Zusammenfassung

Benigne notochordale Tumoren (BNCT) und Chordome sind primäre intraossäre Tumoren, die entlang des Achsenskeletts entstehen und ihre höchste Inzidenz im Sakrum, gefolgt vom Clivus und den thorakalen Wirbelkörpern haben. Neben der klassischen Variante (NOS [„not otherwise specified“] mit hepatoider und nierenzellkarzinom-ähnlichen Varianten) sind eine chondroide Differenzierung sowie Chordome mit polymorpher bis anaplastischer Morphologie beschrieben. Besonders ungünstige Formen sind pädiatrische Chordome mit typischem INI1-Verlust. BNCT und Chordome sind charakterisiert durch folgendes immunhistologisches Profil: Vimentin+, Breitbandzytokeratin+/−, epitheliales membranes Antigen (EMA)+/−, S100-Protein+/+, Brachyury+ und lassen sich so immunhistologisch eindeutig definieren und abgrenzen von Chondrosarkomen, chordoiden Meningiomen und Karzinommetastasen.

Abstract

Benign notochordal tumors (BNCT) and chordomas are primary bone tumors of the spine with a predominant localization in the sacrum and clival region followed by the vertebral bodies. Besides the most common variant (NOS [not otherwise specified] with hepatoid or renal carcinoma cell-like differentiation) chordomas with chondroid, and polymorphic to anaplastic morphology are described. An unfavorable variant are pediatric chordomas with a loss of INI-1. BNCT and chordomas are characterized by the following immunohistological profile: vimentin+, cytokeratin+/−, epithelial membrane antigen (EMA)+/−, S100 protein+/−, brachyury+. This profile helps to distinguish these tumors from other lesions such as chondrosarcoma, chordoid meningioma, and metastases of carcinoma.

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Authors and Affiliations

  1. Institut für Pathologie, Universität Ulm, Ulm, Deutschland

    T. F. E. Barth, A. von Witzleben, P. Möller &  S. Scheil-Bertram

  2. Institut für Pathologie und Zytologie, HELIOS Dr. Horst Schmidt Kliniken Wiesbaden, Ludwig-Erhard-Str. 100, 65199, Wiesbaden, Deutschland

    S. Scheil-Bertram

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  1. T. F. E. Barth
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Correspondence to S. Scheil-Bertram.

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T. F. E. Barth, A. von Witzleben, P. Möller und S. Scheil-Bertram geben an, dass kein Interessenkonflikt besteht.

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

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Barth, T.F.E., von Witzleben, A., Möller, P. et al. Notochordale Tumoren. Pathologe 39, 117–124 (2018). https://doi.org/10.1007/s00292-017-0399-1

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  • DOI: https://doi.org/10.1007/s00292-017-0399-1

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