Given the stagnation in clinical outcomes seen with such conventional therapies over the past 3 decades, a focus of this review is the ongoing efforts to define the genetic and epigenetic changes that are associated with tumor formation and those associated with progression and metastasis. 3 This review summarizes the biological behavior of this tumor and conventional approaches to therapy. The biology of this tumor is most commonly characterized by an appendicular primary tumor with a high rate of metastasis to the lungs. 2 Despite its rarity, however, osteosarcoma represents the most common primary malignancy of bone. In the United States there are approximately 900 new cases of osteosarcoma reported each year. 1 Nonetheless, the biological behavior of and treatments for these distinct subtypes are similar. Within this histological diagnosis, distinct histological subtypes have been defined, including osteoblastic, chondroblastic, fibroblastic, and telangiectatic osteosarcomas. Osteosarcoma is a rare mesenchymal tumor that is histologically characterized by the presence of malignant mesenchymal cells and the production of a bone stroma.
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