Extraskeletal Bone Sarcomas

Extraskeletal bone tumors are rare and high grade tumors including osteosarcoma, chondrosarcoma and Ewing's sarcoma of the soft tissues and its variants. A retrospective study of 25 cases of these tumors was made in our institution in the 1983 - 2000 period. The study of 25 cases revealed that these tumors affect adults (median age 51.2, range 17-73 years). The most common tumor locations were the thigh (12 cases, 48%) and the arm-elbow (6 cases, 24%). As a classification for diagnostic type of tumors: 14 were chondrosarcomas (56%), 8 were osteosarcomas (32%) and 3 were Ewing sarcomas (12%). The median follow-up was 47.07 months with a range from 24 months to 156 months. All the cases were treated with preop. chemotherapy and postoperative radiotherapy (with the exception of the myxoid chondrosarcoma). Globally, the preoperative duration of symptoms ranged from 2 weeks to 6 years (median 6 months). Local recurrences after wide and compartimental margin surgery developed in 7 cases (12%), three cases of chondrosarcoma and four cases of extraskeletal osteosarcoma; and distant metastases developed in seven cases (6 osteosarcomas and 1 Ewing's sarcoma). The two year overall survival rates were: extraeskeletal chondrosarcoma 50%, osteosarcoma 36.5% and Ewing sarcoma 66.6%. The two year disease-free survival was: 42.8% chondrosarcoma, 25% osteosarcoma and 33.3% Ewing sarcoma. The interest of this series is the fact that tumors are high grade and cure may be achieved by wide or compartimental local excision of the tumor at an early stage of the disease (combined with radiation and chemotherapy).