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Bone sarcomas: Preoperative evaluation, histologic classification, and principles of surgical management

Francis J Hornicek, MD, PhD
Section Editors
Alberto S Pappo, MD
Robert Maki, MD, PhD
Raphael E Pollock, MD
Deputy Editors
Diane MF Savarese, MD
Kathryn A Collins, MD, PhD, FACS


Bone sarcomas are malignant spindle cell mesenchymal derived bone tumors, which may or may not produce bone or osteoid. Those tumors which do produce bone are termed osteosarcomas, and there are many different subtypes. (See 'Histologic classification' below.)

Osteosarcoma is the most common primary malignant tumor of bone. Osteosarcomas are characterized by the production of osteoid tissue or immature bone by the malignant cells [1-3]. Osteosarcomas are uncommon tumors compared with carcinomas, with approximately 900 cases diagnosed each year in the United States, mainly in children and adolescents [4]. Among 15 to 29 year olds, bone tumors account for 3 percent of all tumors, and osteosarcoma accounts for about one-half of these cases [5]. Most osteosarcomas present as high-grade tumors and most are located around the anatomic regions of high growth rate.

The survival of patients with malignant bone sarcomas has improved dramatically over the past 30 years, largely as a result of chemotherapeutic advances. Before the era of effective chemotherapy, 80 to 90 percent of patients with osteosarcoma developed metastatic disease despite achieving local control from surgery and died of their disease. It was surmised (and subsequently demonstrated [6]) that the majority of these patients had subclinical metastatic disease that was present at the time of diagnosis, even in the absence of overt metastases.

In osteosarcoma, chemotherapy can successfully eradicate microscopic deposits in the majority of cases if initiated at a time when disease burden is low (ie, following resection of the primary tumor). As a result, all patients with intermediate- or high-grade osteosarcoma receive chemotherapy, although the optimal timing (ie, preoperative or postoperative) is controversial (see 'Adjuvant therapy' below) [7]. Low-grade osteosarcomas, such as parosteal osteosarcomas, are not treated routinely with chemotherapy because the risk of metastatic spread is low.

With modern therapy, approximately two-thirds of patients with non-metastatic extremity osteosarcoma will be long-term survivors, up to 50 percent of those with limited pulmonary metastases may be cured of their disease, and long-term relapse-free survival can be expected in about 25 percent of those who present with metastatic disease overall [8-12].


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