Osteosarcoma is the commonest bone cancer seen in children and adolescents. Its peak incidence is during the adolescent growth spurt, and it occurs more frequently in taller individuals. The commonest site of occurrence is around the knee, ie, distal femur or proximal tibia. The classic presentation consists of pain and swelling at the tumor site. The tumor has a propensity to occur in the metaphyses of long bones, with destruction of normal bone as well as neoplastic new bone formation. Bone scans reveal the tumor.
The standard therapeutic approach has been amputation of the involved extremity with a resection line at least 10 cm above the most proximal extent of the tumor. Amputation alone has yielded a cure rate of approximately 20%. Recently, "limb salvage" (en bloc resection) with removal of the tumorous bone and replacement by a graft or endoprosthetic device has been used. This technique saves the limb but likely does not offer better function in the lower extremity and is associated with an increased risk of local recurrence in the "salvaged" limb.
In the last 10 years, use of adjuvant chemotherapy may have improved the cure rate to approximately 50%, but this result is still being debated. For patients who have a limb amputation, an immediate fitting for a temporary prosthesis is best, later followed by a permanent prosthesis. Psychologic support is extremely important for these youngsters as they not only face the fear of death from cancer, but also of mutilation from therapy.
En bloc resections for osteogenic sarcoma
