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Mesenchymal chondrosarcoma of soft tissues and bone in children, adolescents, and young adults
Author(s) -
Dantonello Tobias M.,
IntVeen Christoph,
Leuschner Ivo,
Schuck Andreas,
Furtwaengler Rhoikos,
Claviez Alexander,
Schneider Dominik T.,
Klingebiel Thomas,
Bielack Stefan S.,
Koscielniak Ewa
Publication year - 2008
Publication title -
cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.052
H-Index - 304
eISSN - 1097-0142
pISSN - 0008-543X
DOI - 10.1002/cncr.23457
Subject(s) - medicine , chondrosarcoma , radiation therapy , surgery , chemotherapy , soft tissue , pelvis , young adult , mesenchymal chondrosarcoma , head and neck
Abstract 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, 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. 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. Cancer 2008. © 2008 American Cancer Society.