If all grades are included, primary fibrosarcoma of the bone has a worse prognosis than osteosarcoma, with a 5-year survival rate of 65%. In high-grade primary fibrosarcoma, the 10-year survival rate is less than 30%. Secondary fibrosarcoma is associated with a very poor outcome, the survival rate at 10 years being less than 10%.
For congenital fibrosarcoma of bone in children, the prognosis (which is related to age and to time to diagnosis) is much better, with the disease having long-term survival rates of higher than 50%.
Soft-tissue fibrosarcoma is associated with a 40-60% survival rate at 5 years. The infantile form has an even better 5-year survival rate, in excess of 80%.
Russell et al reported on four patients with infantile fibrosarcoma treated with chemotherapy and surgical resection, all of whom had excellent functional outcome. [7] The patient with fibrosarcoma of the neck displayed rapid tumor shrinkage. Two of the lower-extremity tumors had only modest changes in dimensions, but on resection, the tumor bed contained fibrous tissue with exaggerated small caliber vessels. In the fourth case, metastatic lesions developed in the central nervous system, orbits, lungs, and kidney after complete removal of the primary tumor.
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Although fibrosarcoma of bone can arise anywhere, it is found most commonly about the knee and femur. The radiograph here shows a typical appearance of a lesion in bone.
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Most pathologists describe the histologic picture of fibrosarcoma as a herringbone pattern. It is an interlacing pattern of sheets of spindle-shaped fibroblasts in a collagen background. This pattern is very distinctive and usually confirms the diagnosis of fibrosarcoma.
