Mesenchymal chondrosarcoma of soft tissues and bone in children, adolescents, and young adults§

Experiences of the CWS and COSS study groups


  • Presented in part at the 38th Congress of the International Society of Paediatric Oncology (SIOP), September 17–21, 2006, Geneva, Switzerland, and awarded with the SIOP best poster prize in the discipline “adolescent and young adult oncology.”

  • We declare that we have no conflict of interest.

  • §

    Involved reference panel members: Oncology: E. Koscielniak, J. Treuner, and S.S. Bielack, Olgahospital, Klinikum Stuttgart, Pediatrics 5 (Oncology, Hematology, Immunology), Germany. N. Graf, University of Homburg, Department of Pediatric Oncology, Germany. H. Jürgens, University Children's Hospital Muenster, Department of Pediatric Hematology and Oncology, Germany. D. Niethammer, University of Tuebingen, Department of Pediatric Oncology, Germany. K. Winkler, University of Hamburg, Department of Pediatric Oncology, Germany. Pathology:H. Bürger, University of Muenster, Department of Pathology, Germany. G. Delling, University of Hamburg, Institute of Osteopathology, Germany. D. Harms, U. Jänig, and I. Leuschner, University of Kiel, Department of Pediatric Pathology, Germany. G. Jundt, University of Basel, Department of Pathology, Switzerland. P. Meister, University of Munich, Department of Pathology, Germany. A. Schulz, University of Giessen, Department of Pathology, Germany. Radiology: P. Winkler, Olgahospital, Klinikum Stuttgart, Department of Pediatric Radiology, Germany. Radiotherapy: B.F. Schmidt, Katharinenhospital, Klinkum Stuttgart, Department of Radiotherapy, Germany. A. Schuck, University of Muenster, Department of Radiotherapy, Germany. R. Schwarz, University of Hamburg, Department of Radiotherapy. Surgery: G. Gehrke, Henriettenstiftungskrankenhaus, Department of Maxillofacial Surgery, Hannover, Germany. U. Heise, Hamburg, Germany. R. Schmelzle, University of Hamburg, Department of Maxillofacial Surgery, Germany. W. Winkelmann, University of Muenster, Department of Orthopedics, Germany

  • Centers contributing patients: University Hospital Bonn, Germany. Carl-Thiem-Klinikum Cottbus, Germany. Klinikum Dortmund, Germany. University Hospital, Ulm, Germany. University Hospital Duesseldorf, Germany. University Hospital Greifswald, Germany. University Hospital Hamburg, Germany. University Hospital Heidelberg, Germany. University Hospital Jena, Germany. University Hospital Kiel, Germany. Olgahospital, Klinikum Stuttgart, Germany. University Hospital Tuebingen, Germany



Mesenchymal chondrosarcoma (MCS) is a rare tumor with a strong tendency toward late recurrences leading to reported 10-year survival rates below 50%. The recommended treatment is resection with wide margins; the effectiveness of chemo- and radiotherapy remain poorly defined. As reports about MCS in young patients are scarce, treatment and outcomes of children/adolescents/young adults in the CWS and COSS studies were investigated.


Since 1977, 15 of >7000 CWS and COSS patients ≤25 years had a confirmed diagnosis of MCS.


The median age was 16.6 (range, 1–25) and median follow-up 9.6 years (range, 1–22). Four MCS were osseous and 11 extraosseous. All but 1 patient had nonmetastatic disease. Tumor sites were head/neck (n = 6), paravertebral (n = 3), pelvis (n = 3), limbs (n = 2), and kidney (n = 1). All tumors were resected, but only 8 completely. Thirteen individuals received chemotherapy, 6 were irradiated. Actuarial 10-year event-free and overall survival rates were 53% and 67%, respectively. Four recurrences occurred, all within 4 years from diagnosis (3 local, 1 combined; 2 of these in irradiated patients). One of these patients survived after surgery and radiation for local recurrence. Seven of 8 patients whose tumors were completely resected during primary treatment, but only 4 of 7 patients with incomplete surgery survived disease-free.


This series of 15 MCS differs from others: the median age was young, most tumors were extraosseous, most patients received chemotherapy, and the outcome was better than published. Despite long-term follow-up, characteristic late metastatic recurrences were not observed. Treating MCS according to standard multimodal soft tissue/bone sarcoma regimens is proposed. Cancer 2008. © 2008 American Cancer Society.