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Primäres extraaxiales chondroides Chordom des anterioren Nasenseptums: Fallbericht eines seltenen Chordoms mit Literaturübersicht

Primary extra-axial chondroid chordoma of the anterior nasal septum: case report of a rare chordoma with literature review

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Zusammenfassung

Ein 87-jähriger Patient berichtete über eine knotige, größenprogrediente Struktur des anterioren Nasenseptums mit partieller Verlegung der Naseneingänge. In der Bildgebung sowie intraoperativ zeigte sich die Raumforderung lokal begrenzt, ohne Anzeichen der Infiltration von Subkutis, Knochen sowie der angrenzenden Sinus paranasales. Histologisch zeigte sich ein chondroider Tumor mit lobulärem Wachstum sowie physaliphorem Zytoplasma. Durch die immunhistochemische Untersuchung konnte bei Positivität für Brachyury und Negativität für ein EWSR1(Ewing-Sarcoma-Breakpoint-Region)-Rearrangement die Diagnose eines chondroiden Chordoms gestellt werden. Der vorliegende Fall belegt die differenzialdiagnostischen Überlegungen zu diesem Tumor, der per se selten ist, aber auch an untypischen extraaxialen Stellen lokalisiert sein kann. Nach Literaturrecherche wurde eine Übersicht aller von uns identifizierten 34 primären extraossären Chordome der Nase, des Nasopharynx und der paranasalen Sinus erstellt und der vorgestellte Fall in diese Übersicht eingeordnet.

Abstract

An 87-year-old patient reported a nodular, progressively enlarging mass of the anterior nasal septum leading to partial obstruction of the nostrils. The tumor showed no infiltration of the subcutis, bone, or paranasal sinuses in imaging or intraoperatively. Histological examination revealed a chondroid tumor with lobular growth and physaliferous cell morphology. Immunohistochemistry revealed a brachyury-positive tumor without EWSR1 rearrangement, leading to the diagnosis of a chondroid chordoma. The reported case demonstrates the differential diagnostic considerations pertaining to this rare tumor, which can also have an untypical and very rare extra-axial location. Review of the literature identified 34 primary extraosseous chordomas of the nose, nasopharynx, and paranasal sinuses, and allowed the nasal chordoma presented herein to be included in this group of extra-axial chordomas.

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Authors and Affiliations

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

    C. Holley, P. Möller & T. F. E. Barth

  2. Klinik für Diagnostische und Interventionelle Radiologie, Universitätsklinikum Ulm, Ulm, Deutschland

    T. Breining

  3. Klinik für Hals‑, Nasen‑, Ohrenheilkunde, Kopf- und Halschirurgie, Universitätsklinikum Ulm, Ulm, Deutschland

    M. Scheithauer

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  1. C. Holley
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  4. P. Möller
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  5. T. F. E. Barth
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Correspondence to C. Holley.

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Interessenkonflikt

C. Holley, T. Breining, M. Scheithauer, P. Möller und T.F.E. Barth geben an, dass kein Interessenkonflikt besteht.

Für diesen Beitrag wurden von den Autoren keine Studien an Menschen oder Tieren durchgeführt. Für die aufgeführten Studien gelten die jeweils dort angegebenen ethischen Richtlinien. Für Bildmaterial oder anderweitige Angaben innerhalb des Manuskripts, über die Patienten zu identifizieren sind, liegt von ihnen und/oder ihren gesetzlichen Vertretern eine schriftliche Einwilligung vor.

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Holley, C., Breining, T., Scheithauer, M. et al. Primäres extraaxiales chondroides Chordom des anterioren Nasenseptums: Fallbericht eines seltenen Chordoms mit Literaturübersicht. HNO 69, 221–228 (2021). https://doi.org/10.1007/s00106-020-00957-3

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  • DOI: https://doi.org/10.1007/s00106-020-00957-3

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