Osteosarcoma results from the dysregulated growth of osteoblasts -- the cells that form the bone matrix. It primarily develops near the ends of the femur, tibia or humerus and is usually diagnosed during adolescence.

While the precise causes of osteosarcoma are unknown, scientists said it's evident two tumor suppressor genes -- p53 and Rb -- are involved, as children with familial mutation syndromes affecting either of those genes have higher incidences of osteosarcoma.

Dr. Stuart Orkin of the Dana-Farber Cancer Institute and colleagues developed a novel experimental system to model the genetics of human osteosarcoma. The researchers said they concluded p53 loss is essential for the development of osteosarcoma, and that while Rb gene mutation acts synergistically with p53 loss to facilitate carcinogenesis, loss of Rb alone is not sufficient to induce osteosarcomagenesis.

Orkin said he's hopeful the findings "will stimulate translational efforts to develop novel therapies for this devastating bone tumor."

The research is reported in the journal Genes & Development.