Sarcoma

Tumors: primary malignant and bone and soft tissue

Primary malignant bone and soft tissue tumors are a rare condition in children. They account for less than 3000 new cases annually in the US. They account for approximately 11% of all tumors. Despite this, malginant tumors of bone account for more than 50% of amputations performed in children. It is also one of the most common causes of death among adolescents. Currently, 80 to 85% of the patients with primary bone tumors can be managed safely with wide resection and limb preservation without affecting their long-term outcome.

Advances in imaging and chemotherapeutic agents have dramatically increased the long-term survival of sarcoma patients. MRI has improved our ability to visualize the tumor and evaluate its response to chemotherapy. Chemotherapy and radiotherapy have resulted in an increased survival rate. With preoperative chemotherapy along with surgery the five year survival rate for osteosarcoma and Ewing's sarcoma approaches 70 to 80%. The prognostic implications and treatment significance of genetic advancements are still evolving, but will most likely play a major role in the near future. 

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