Elucidation of the molecular mechanisms of malignant bone tumors: refining diagnosis and identifying novel targets for treatment.

Overview

Primary bone tumors are rare, accounting for less than 0.2% of all cancers diagnosed yearly in the United States. Study of the molecular mechanisms of these diseases has given insight into their pathobiology. It has also identified molecular alterations that, if present, may be used in conjunction with histologic evaluation to further refine diagnosis, allowing cases to be stratified into prognostic groups more or less likely to show response to current cytotoxic protocols. Recent findings have lead to the identification of molecular pathways that may serve as targets of novel therapies, especially in the case of Ewing sarcoma. Telomere maintenance mechanisms are also emerging as potential targets for anticancer therapy. As the molecular mechanisms underlying malignant bone tumors are better understood, new anticancer agents targeting specific pathways are likely to emerge. This may make it possible to tailor treatment for each individual patient, using a combination of cytotoxic and targeted therapies based on the histologic and molecular profile of the patient's tumor.