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Extraskeletal osteosarcoma has a favourable prognosis when treated like conventional osteosarcoma

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Abstract

Purpose: The aims of this analysis were to investigate the clinical features of extraskeletal osteosarcoma (ESOS) and examine the outcome after multi-modal therapy. Methods: The co-operative osteosarcoma study-group database was searched for patients with extraskeletal osteosarcoma. Eligible patients were included in a retrospective analysis of patient, tumour and treatment related variables and outcome. As for conventional osteosarcoma, scheduled treatment included surgery and multi-agent chemotherapy. Results: Seventeen eligible patients were identified with a median age of 44 years (range, 3–65 years). The thigh was the commonest tumour site. Two patients had a history of previous malignancies and two had primary metastases. Median follow-up was 3.2 years (range: 0.6–7.4 years) and at last follow-up, 11 patients were alive in complete remission, 3 patients were alive with disease and 3 patients had died of their disease. Three-year overall actuarial and event-free survival rates were 77% and 56%, respectively. Patients with macroscopically complete surgical remission had an improved overall survival (P=0.0004). Conclusions: The patients in this retrospective study had a surprisingly good survival rate. This may be due to the combination of multi-agent chemotherapy with surgery, and we recommend this approach in the treatment of ESOS.

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Acknowledgements

The studies that form the basis of this work were supported by Deutsche Krebshilfe, Bundesministerium für Forschung und Technologie and Fördergemeinschaft Kinderkrebszentrum Hamburg. S.Y. Goldstein-Jackson received support from the European Community action scheme for the mobility of university students (Erasmus). The authors wish to thank all patients who contributed to the COSS studies and to acknowledge the physicians, nurses, data managers and support staff of the collaborating centres for their active participation. They would like to thank Mathias Kevric for his expert data management. The patients included in this study were from the following hospitals: Universitätsklinikum Münster, Medizinische Klinik und Poliklinik A, Germany; Kinder-Poliklinik, Päd. Onkologie/KMT, Munich; Germany; Medizinische Universitätsklinik, Abteilung Onkologie/Hämatologie, Hamburg, Germany; St. Anna Kinderspital, Vienna, Austria; Medizinische Universitätsklinik I, Cologne, Germany; Medizinische Universitätsklinik, Abteilung Onkologie/Hämatologie, Graz, Austria; Orthopädische Universitätsklinik Balgrist, Zürich, Switzerland; Katharinen – Hospital, Stuttgart; Germany; Medizinische Klinik& Poliklinik V, Heidelberg, Germany; Medizinische Universitätsklinik, Würzburg, Germany; Mutterhaus der Borromäerinnen, Trier, Germany; Medizinische Universitätsklinik I, Abt.: Hämatologie/Internistische Onkologie, Regensburg, Germany; Medizinische Klinik II mit Schwerpunkt Onkologie und Hämatologie, Charité Campus Mitte, Berlin, Germany; Universitätsklinikum Schleswig-Holstein, II. Medizinische Klinik und Poliklinik, Kiel, Germany; Kantonspital Aarau, Abteilung Pädiatrische Onkologie, Aarau, Switzerland; Diakonissenkrankenhaus, Medizinische Klinik II, Stuttgart, Germany; Medizinische Universitätsklinik, Abteilung Hämatologie/Onkologie, Rostock, Germany

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Authors and Affiliations

  1. Klinik und Poliklinik für Kinder- und Jugendmedizin, Pädiatrische Hämatologie und Onkologie, Universitätsklinikum Münster, Münster, Albert-Schweitzer-Str. 33, 48149, Germany

    Sing Yu Goldstein-Jackson, Jan-Nicolas Machatschek, Heribert Jürgens & Stefan S. Bielack

  2. University of Wales College of Medicine, Heath Park, Cardiff, Wales, United Kingdom

    Sing Yu Goldstein-Jackson

  3. Klinik und Poliklinik für Allgemeine Orthopädie, Universitätsklinikum Münster, Münster, Germany

    Georg Gosheger

  4. Institut für Osteopathologie, Universitätsklinikum Hamburg-Eppendorf, Hamburg, Germany

    Günter Delling

  5. Medizinische Klinik und Poliklinik A, Universitätsklinikum Münster, Münster, Germany

    Wolfgang E. Berdel

  6. Orthopädische Universitätsklinik Balgrist, Zürich, Switzerland

    G. Ulrich. Exner

  7. Knochentumor-Referenzzentrum am Institut für Pathologie, Universitätsspital Basel, Basel, Switzerland

    Gernot Jundt

  8. St. Anna Kinderspital, Vienna, Austria

    Andreas Zoubek

Authors
  1. Sing Yu Goldstein-Jackson
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  2. Georg Gosheger
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  3. Günter Delling
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  4. Wolfgang E. Berdel
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  5. G. Ulrich. Exner
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  6. Gernot Jundt
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  7. Jan-Nicolas Machatschek
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  8. Andreas Zoubek
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  9. Heribert Jürgens
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  10. Stefan S. Bielack
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for the Cooperative Osteosarcoma Study Group COSS

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Correspondence to Stefan S. Bielack.

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Goldstein-Jackson, S.Y., Gosheger, G., Delling, G. et al. Extraskeletal osteosarcoma has a favourable prognosis when treated like conventional osteosarcoma. J Cancer Res Clin Oncol 131, 520–526 (2005). https://doi.org/10.1007/s00432-005-0687-7

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  • DOI: https://doi.org/10.1007/s00432-005-0687-7

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