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Leiomyosarcoma of the inferior vena cava extending into the right atrium

A rare differential diagnosis of a right atrial tumor with fatal outcome

Leiomyosarkom der V. cava inferior mit Ausdehnung bis in den rechten Vorhof

Seltene Differentialdiagnose eines rechtsatrialen Tumors mit letalem Ausgang

  • Case study
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Abstract

A 54-year-old female patient presented with a progressive and deteriorating dyspnea at the slightest exertion in particular during the past few days before presentation. Transthoracic echocardiography revealed a large space-occupying lesion in the right atrium extending into the inferior vena cava (IVC). Abdominal magnetic resonance aortography showed an elongated space-occupying lesion in the IVC with a significant portion of the tumor and almost completely filling the right atrium accompanied by an infiltration of the hepatic and renal veins. A pronounced tumor infiltration of the IVC at the level of the liver was confirmed intraoperatively and immunohistochemical analysis showed a moderate to poorly differentiated leiomyosarcoma. The extended tumor was successfully removed by a complex operation of the thorax and abdomen but the procedure was accompanied by severe bleeding. A few hours following the procedure the patient died due to a further episode of irreversible intra-abdominal hemorrhage.

Zusammenfassung

Eine 54-jährige Patientin stellte sich wegen einer progressiven und in den letzten Tagen bei geringster Belastung bestehenden Dyspnoe vor. Im transthorakalen Echokardiogramm zeigte sich eine große Raumforderung im rechten Vorhof, die bis in die V. cava inferior (VCI) reichte. In einer Magnetresonanzaortographie des Abdomens fanden sich eine längliche Raumforderung in der VCI mit großem Tumoranteil bis in den rechten Vorhof reichend, sodass der rechte Vorhof fast komplett ausgefüllt wurde, sowie eine begleitende Infiltration der Leber- und Nierenvenen. Intraoperativ zeigte sich eine ausgeprägte Infiltration der VCI auf Höhe der Leber durch diesen Tumor. Immunhistochemisch stellt sich das Bild eines teils mäßig, teils gering differenzierten Leiomyosarkoms dar. Der ausgedehnte Tumor konnte in einem komplexen Zweihöhleneingriff, der mit sehr viel Blutverlust einherging, entfernt werden. Die Patientin verstarb jedoch einige Stunden nach diesem operativen Eingriff an einer erneuten unstillbaren Blutung.

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Conflict of interest

The corresponding author states that there are no conflicts of interest.

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Authors and Affiliations

  1. Department of Internal Medicine, DRK Hospital Sondershausen, Hospitalstr. 2, 99706, Sondershausen, Germany

    U. Lotze & J. Reponova

  2. Department of Internal Medicine, DRK Hospital Sömmerda, Sömmerda, Germany

    G. Muth & G. Oltmanns

  3. Joint Practice for Radiology (DM H.-C. Reich/Dr. G. Etzrodt/Dr. R. Górna), Sondershausen, Germany

    H.-C. Reich & G. Etzrodt

  4. Institute of Diagnostic and Interventional Radiology, University Hospital of Jena, Jena, Germany

    W.A. Kaiser

  5. Department of Internal Medicine I, University Hospital of Jena, Jena, Germany

    O. Mutschke

  6. Institute of Pathology, University Hospital of Cologne, Cologne, Germany

    M Ortmann

  7. Department of General, Visceral and Cancer Surgery, University Hospital of Cologne, Cologne, Germany

    D. Stippel

  8. Department of Cardiothoracic Surgery, University Hospital of Cologne, Cologne, Germany

    T. Wahlers

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  1. U. Lotze
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  2. J. Reponova
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  3. G. Muth
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  4. G. Oltmanns
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  5. H.-C. Reich
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  6. G. Etzrodt
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  7. W.A. Kaiser
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  8. O. Mutschke
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  9. M Ortmann
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  10. D. Stippel
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  11. T. Wahlers
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Corresponding author

Correspondence to U. Lotze.

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Lotze, U., Reponova, J., Muth, G. et al. Leiomyosarcoma of the inferior vena cava extending into the right atrium. Herz 37, 573–578 (2012). https://doi.org/10.1007/s00059-011-3580-y

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  • DOI: https://doi.org/10.1007/s00059-011-3580-y

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