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Osteosarcoma is the most common malignant primary bone tumor in children and adolescents.Osteosarcoma’s destruction of normal bone architecture and its production of malignant osteoid can permit its identification from skeletal remains, allowing the recognition that it is an ancient disease. Among the earliest malignant bone tumor described in humans, it was presumed to be identified in the humerus of a male Celt from 800 to 600 BC.1 A probable osteosarcoma dates back to 250 AD in the pelvis of a young individual from Ancient Egypt. A definite osteosarcoma with a classic sunburst appearance was found in the remains of the femur of a Peruvian from approximately 1100 to 1200 AD.1 The term osteosarcoma was introduced by Alexis Boyer (1757–1833), and the first large clinical-pathological description of sarcomas of bone was performed by Samuel Weissel Gross (1837–1889).2 In this description, the tendency for hematogenous and not lymphatic dissemination, as well as the occurrence of skip metastases, led to the suggestion that amputation be performed at a distance beyond the primary lesion.2

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The diagnosis of osteosarcoma can be made based on histologic examination of pathologic material, staging can be performed with imaging procedures, and multimodality therapy including chemotherapy and surgery successfully treat the majority of patients. Much information has been acquired about the natural history and biology of this disease. Despite all of these advances, osteosarcoma remains enigmatic. The biology of the tumor is incompletely understood, and recent trials modifying therapy have failed to produce further advances. Patients with localized disease have a 5-year survival of at least 70%; patients with metastatic or recurrent disease have a < 20% chance of long-term survival despite aggressive therapies. These figures have changed little in the past 2 decades.3-6 A challenge over the next few years will be to develop strategies to understand better the biology and behavior of this tumor, to facilitate further advances in its treatment. The objective of this chapter is to review what is known about osteosarcoma, including its epidemiology, etiology, biology, presentation, evaluation, staging, pathology, treatment, and future approaches. This will establish a context in which emerging strategies are being developed which may change the outlook for patients with this disease.

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Epidemiology/Etiology

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Several features characterize the epidemiology of osteosarcoma and have been taken as providing some clues as to its pathogenesis. Osteosarcoma has a bimodal age distribution with the first peak in the second decade of life and the second among older adults. The approximate incidence of osteosarcoma is 4.8 per million children younger than 20 years of age.7 There is some variability in the annual incidence rate as shown in eFigure 453.1. The incidence by age is as low as 0.3 per million in children under the age of 5 years, 2.8 per million between the ages of 5 and 9 years, 8.3 per million between the ages of 10 and 14 years, and 9.4 per million between the ages of 15 and 19 years.7 The incidence by age at diagnosis is shown in eFigure ...

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