Osteosarcoma – An Evaluation of Current Diagnosis, Treatment and Chemotherapy

Bahtiyar Demiralp, Avudaiappan Maran, Scott H Okuno, Gobinda Sarkar, Michael J Yaszemski
European Musculoskeletal Review, 2011;6(1):18-23
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Abstract

Abstract
Osteosarcoma is a bone tumour that most often affects children and young adults. Although a combination of surgery to remove the primary tumour and chemotherapy prior to and after the surgery has led to an improved survival rate, local recurrence and metastases still develop in two-fifths of patients. A definitive therapy is yet to be determined for this deadly disease. This article discusses the current status on diagnosis and treatment, with an emphasis on developing new molecularly targeted therapies.

Keywords
Bone tumour, chemotherapy, osteosarcoma, sarcoma, tumour surgery, 2-methoxyestradiol

Disclosure: The authors have no conflicts of interest to declare.
Received: 6 May 2010 Accepted: 10 December 2010 Citation: European Musculoskeletal Review, 2011;6(1):18–23
Correspondence: Avudaiappan Maran, Mayo Clinic, 200 First Street SW, MS 3-69, Rochester MN 55905, US. E: maran.avudaiappan@mayo.edu

Osteosarcoma is the most common malignant primary bone tumour in children and adolescents. It is the sixth leading cancer in children >15 years of age. There are approximately 400 new cases each yearin the US.1,2 Osteosarcoma affects males more frequently than females, with a ratio of 1.6:1. It occurs early in females due to the earlier onset of growth spurt.3–5 The second peak for osteosarcoma is between the sixth and eighth decades. The pathogenesis is still unclear, although several factors have been proposed. The standardclinical treatment consists of a combination of surgery and chemotherapy. There is limited knowledge, however, on how different types of surgery (limb salvage versus amputation), chemotherapy (pre- and post-operative) or other treatments (e.g. radiotherapy) influence the outcome.1

Osteosarcoma has been reported to occur in all bones of the body. It has an affinity with the metaphyseal portions of the long bones. It is commonly localised in the distal femur and proximal tibia region, which was observed in 973 out of 1,649 cases in a study carried out at the Mayo Clinic in Rochester.6 These sites generally contain growth plates with higher proliferative activity and turnover of bone.7 The next most common location is the proximal humerus. It also rarely affects the bones of the hands and wrists.

In the group of axial locations of osteosarcoma, pelvic osteosarcomas account for approximately 7–9% of all osteosarcomas.3 Spine osteosarcomas occur in 0.85–3% of cases.9 Osteosarcoma occasionally arises in the soft tissue, thyroid gland, heart, kidney, uterus and lung.3,10

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