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Primär maligne Knochentumoren

Primary malignant bone tumors

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Zusammenfassung

Unter der Vielzahl möglicher Tumorerkrankungen sind primäre Neoplasien des Skeletts vergleichsweise selten. Primär maligne Knochentumoren zeigen eine jährliche Inzidenz von knapp 10 Erkrankungen pro 1 Mio. Einwohner. Im Kindesalter ist der Anteil maligner Knochentumoren mit 6% aller kindlichen Malignome größer als der entsprechende Anteil bei Erwachsenen. Der Altersgipfel der Gesamtheit aller Knochentumoren liegt zwischen 15 und 19 Jahren. Am häufigsten sind das Osteo- (35%), das Chondro- (25%) und das Ewing-Sarkom (16%). Seltener (≤ 5%) folgen Entitäten wie das Chordom, das primäre maligne fibröse Histiozytom (MFH) des Knochens und das Fibrosarkom des Knochens. Vaskuläre primäre maligne Knochentumoren und das Adamantinom sind sehr selten. Das für eine systematische Therapieableitung notwendige Staging beinhaltet eine vollständige bildgebende Diagnostik und die histopathologische Diagnosesicherung der Entität, welche im Fall von malignen Knochentumoren meist über eine Probebiopsie erfolgt. Darauf basierend muss in einer interdisziplinären Tumorkonferenz das individuelle therapeutische Vorgehen festgelegt werden. Die operative Therapie beinhaltet dabei zumeist die weite Tumorresektion und anschließende Rekonstruktion. Die engmaschige, kontinuierliche Tumornachsorge ist von essenzieller Bedeutung für die Prognose und sollte üblicherweise im behandlungsführenden Tumorzentrum fortgeführt werden.

Abstract

Among human neoplasms, primary malignant bone tumors are fairly rare. They present an incidence rate of roughly 10 cases per 1 million inhabitants per year. During childhood (<15 years), the percentage of malignant bone tumors amounts to 6% of all infantile malignancies. Only leukemia and lymphoma show a higher incidence in adolescence. Of all primary malignant bone tumors, 60% affect patients younger than 45 years and the peak incidence of all bone tumors occurs between 15 and 19 years. The most common primary malignant bone tumors are osteosarcoma (35%), chondrosarcoma (25%), and Ewing’s sarcoma (16%). Less frequently (≤ 5%) occurring tumors are chordoma, malignant fibrous histiocytoma of bone, and fibrosarcoma of bone. Vascular primary malignant tumors of bone and adamantinoma are very rare. Staging of the lesion is essential for systemic therapeutic decision-making and includes complete imaging and histo-pathological confirmation of the suspected entity. In most cases, this is established by open- or image-guided biopsy. Based on this information, an interdisciplinary tumor board will determine the individual therapeutic approach. Endoprosthetic or biological reconstruction following wide tumor resection is the most common surgical therapy for primary malignant bone tumors. There is vital importance in a thorough postoperative follow-up and continous after-care by a competent tumor center which is permanentely in charge of therapy.

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  1. Klinik für Orthopädie und Sportorthopädie, Klinikum rechts der Isar, Technische Universität München, Ismaninger Straße 22, 81675, München, Deutschland

    R. von Eisenhart-Rothe, A. Toepfer, J. Schauwecker, H. Gollwitzer & H. Rechl

  2. Kinderorthopädische Klinik, Behandlungszentrum Aschau, Aschau, Deutschland

    M. Salzmann

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  1. R. von Eisenhart-Rothe
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  2. A. Toepfer
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  3. M. Salzmann
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von Eisenhart-Rothe, R., Toepfer, A., Salzmann, M. et al. Primär maligne Knochentumoren. Orthopäde 40, 1121–1142 (2011). https://doi.org/10.1007/s00132-011-1866-7

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