Mesenchymal chondrosarcoma of soft tissues and bone in children, adolescents, and young adults - Experiences of the CWS and COSS study groups

被引:87
作者
Dantonello, Tobias M. [1 ]
Int-Veen, Christoph [1 ]
Leuschner, Ivo [2 ]
Schuck, Andreas [3 ]
Furtwaengler, Rhoikos [4 ]
Claviez, Alexander [5 ]
Schneider, Dominik T. [6 ,7 ]
Klingebiel, Thomas [8 ]
Bielack, Stefan S. [1 ,9 ]
Koscielniak, Ewa [1 ,10 ]
机构
[1] Klinikum Stuttgart, Olgahosp, D-70176 Stuttgart, Germany
[2] Univ Kiel, Inst Pediat Pathol, Kiel, Germany
[3] Univ Munster, Dept Radiotherapy, Munster, Germany
[4] Saarland Univ Hosp, Dept Pediat Oncol, Homburg, Germany
[5] Univ Kiel, Dept Pediat Oncol, Kiel, Germany
[6] Univ Duesseldorf, Dept Pediat Oncol, Dusseldorf, Germany
[7] Pediat Clin, Dortmund, Germany
[8] Univ Frankfurt, Dept Pediat Oncol, Frankfurt, Germany
[9] Univ Childrens Hosp Muenster, Dept Pediat Hematol & Oncol, Munster, Germany
[10] Univ Tubingen, Dept Pediat Oncol, Tubingen, Germany
关键词
pediatric oncology; adolescent oncology; soft tissue sarcoma; bone sarcoma; mesenchymal chondrosarcoma;
D O I
10.1002/cncr.23457
中图分类号
R73 [肿瘤学];
学科分类号
100214 ;
摘要
BACKGROUND. 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. METHODS. Since 1977, 15 of > 7000 CWS and COSS patients <= 25 years had a confirmed diagnosis of MCS. RESULTS. 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, I 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. CONCLUSIONS. 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.
引用
收藏
页码:2424 / 2431
页数:8
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