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Handchir Mikrochir Plast Chir 2000; 32(4): 283-290
DOI: 10.1055/s-2000-10930
DOI: 10.1055/s-2000-10930
Fallbericht
Georg Thieme Verlag Stuttgart · New York
Maligner Verlauf einer pigmentierten villonodulären Synovialitis der Beugesehnenscheide des Kleinfingers - Fallbericht und Literaturübersicht[*]
Malignant Pigmented Villonodular Synovialitis of Flexor Tendon Sheath of Little FingerFurther Information
Publication History
Publication Date:
31 December 2000 (online)
Zusammenfassung
Es wird über einen 55-jährigen Mann berichtet, bei dem unter dem klinischen Bild eines chronischen Sehnenscheideninfektes im Bereich des Kleinfingers eine Inzision erfolgte, bei der sich anstelle von Eiter oder Wundsekret nur bräunlich-sulziges Gewebe entleerte. Aus der Biopsie wurde die Diagnose einer pigmentierten villonodulären Synovialitis (PVNS) der Sehnenscheide gestellt. Das Röntgenbild des Fingers wies eine umschriebene Osteolyse auf. Die nachfolgende Abklärung im MRT zeigte eine Ausbreitung auf die Hand und den Unterarm im Verlauf der Beugesehnen. Eine Strahlen-Therapie brachte zunächst einen klinischen Befundrückgang. In der Folgezeit fanden sich Lungenmetastasen, eine diffuse Destruktion des Handskelettes, die zur Unterarmamputation führte, und eine Zungenmetastase. Der Patient verstarb an einer pulmonalen Insuffizienz bei einer Metastasenlunge. In Verbindung mit der pathomorphologischen Sektion wurde der Tumor abschließend als unklassifizierbares metastasierendes Sarkom beurteilt. Dem eigenen Fall werden die seltenen in der Literatur übermittelten Fälle einer malignen PVNS, ausgehend von Sehnenscheiden oder Gelenken, gegenübergestellt.
Summary
The case of a 55-year-old patient is described, presenting the clinical sign of a chronic infection of the tendon sheath. Upon incision a mass of brown synovial tumor was found. The X-ray showed a circumscript bone erosion. MRI demonstrated tumor involvement of all flexor tendons up to the forearm. Under radiation the tumor initially was diminished. Half a year later, pulmonary metastases were found. The destruction of the whole skeleton of the hand led to a forearm amputation. Later, a metastasis was found in the tongue. The patient died with the clinical signs of pulmonary insufficiency. Autopsy showed diffuse pulmonary metastases. This case is discussed together with other rare cases of malignant pigmented villonodular synovialitis arising from joints and tendon sheaths.
Schlüsselwörter
Tumoren - Sehnenscheide - pigmentierte villonoduläre Synovialitis
Key words
Malignant tumor of tendon sheath - malignant pigmented villonodular synovialitis
1 Nach einem Vortrag auf dem 40. Symposium der Deutschsprachigen Arbeitsgemeinschaft für Handchirurgie vom 20. bis 23. Oktober 1999 in Hannover.
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1 Nach einem Vortrag auf dem 40. Symposium der Deutschsprachigen Arbeitsgemeinschaft für Handchirurgie vom 20. bis 23. Oktober 1999 in Hannover.
Dr. med. Jiri Huracek
Abteilung für Hand- und periphere NervenchirurgieKlinik für Wiederherstellende ChirurgieKantonsspital
Spitalstrasse 21
4031 Basel
Schweiz