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Fallbericht eines high-grade Osteosarkoms der Kieferhöhle

High-grade osteosarcoma of the maxillary sinus. A case report

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Zusammenfassung

Hintergrund

Das Kieferostesosarkom (KOS), das 5–13% aller Osteosarkome ausmacht, ist ein lokal aggressiv wachsender, zu Lokalrezidiven neigender maligner mesenchymaler Tumor, der sich durch die Bildung von Tumorosteoid auszeichnet. Im Vergleich zu Osteosarkomen des übrigen Skeletts (SOS) metastasiert das KOS relativ selten und relativ spät. Es unterscheidet sich in seinem biologischen Verhalten signifikant vom SOS.

Fallbericht

Im dargestellten Fall geht es um einen 60-jährigen Patienten mit einer neu aufgetretenen polypösen Schleimhautwucherung am rechten Oberkieferalveolarfortsatz, die sich bioptisch zunächst als Granulationsgewebspolyp herausstellte. Nach transmaxillärer Tumorausräumung und Konsultation eines Knochentumor-Referenzzentrums konnte die endgültige Diagnose eines hochmalignen chondroblastischen Osteosarkoms gestellt werden. Die empfohlene radikale Tumorresektion wurde vom Patienten wegen der zu erwartenden kosmetischen Einbußen abgelehnt. Nach chirurgischer Reduktion des foudroyant wachsenden Sarkoms wurde bei CT-graphisch nachweisbarer diffuser pulmonaler Metastasierung eine palliative Chemotherapie nach dem COOS-/EURO-B.O.S.S.-Protokoll eingeleitet, die den Krankheitsprogress nur temporär aufhalten konnte. Der Patient verstarb an progredienter respiratorischer Insuffizienz.

Schlussfolgerung

Die radikale chirurgische Resektion ist beim KOS die Therapie der Wahl. Zur Diagnosesicherung und zentralen Erfassung der Fälle sollte ein Knochentumor-Referenzzentrum eingeschaltet werden.

Abstract

Background

Osteosarcoma of the jaw (JOS) constituting 5% to 13% of all osteosarcoma is a locally aggressive malignant mesenchymal tumor with high tendency to local recurrence and the ability to produce tumor osteoid. Compared to osteosarcoma of the remaining skeleton (SOS) JOS metastasizes relatively rarely and relatively late. It differs significantly from SOS in its biological behaviour.

Case report

The presented case deals with a 60-year-old male patient suffering from a newly occurred polypous mucosal tumor of the right-sided maxillary alveolar ridge bioptically diagnosed as a granulation tissue polyp first. After transmaxillary resection of the tumor and consultation of a bone tumor reference center the final diagnosis of a high-grade chondroblastic osteosarcoma could be made. The recommended radical resection of the tumor was declined by the patient because of the expected cosmetic consequences. After surgical reduction of the rapidly growing sarcoma chemotherapy according to the COOS/EURO-B.O.S.S. protocol was initiated at a stage when computed tomography showed diffuse metastatic disease to the lungs. Chemotherapy could delay the progress of the disease only temporarily. The patient died from respiratory insufficiency.

Conclusion

In JOS radical surgical resection is the therapy of first choice. For substantiation of the diagnosis and central registration of the cases a bone tumor reference center should be contacted.

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Authors and Affiliations

  1. Klinik für Zahn-, Mund-, Kiefer- und Plastische Gesichtschirurgie des Universitätsklinikums der RWTH Aachen, Pauwelsstraße 30, 52074, Aachen, Germany

    Marcus Gerressen, Siegfried Jänicke, Ralf Smeets, Dieter Riediger & Alireza Ghassemi

  2. Institut für Pathologie des Universitätsklinikums, RWTH Aachen, Aachen, Germany

    Andreas Donner

  3. Knochentumor-Referenzzentrum der Schweizerischen Gesellschaft für Pathologie, Universitätsklinikum Basel, Basel, Switzerland

    Gernot Jundt

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  1. Marcus Gerressen
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  2. Andreas Donner
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  3. Gernot Jundt
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  4. Siegfried Jänicke
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  6. Dieter Riediger
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  7. Alireza Ghassemi
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Correspondence to Marcus Gerressen.

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Gerressen, M., Donner, A., Jundt, G. et al. Fallbericht eines high-grade Osteosarkoms der Kieferhöhle. Mund Kiefer GesichtsChir 10, 347–352 (2006). https://doi.org/10.1007/s10006-006-0021-9

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