The Role of Animal Models in Determining the Genetic Bases of Osteosarcoma

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Osteosarcoma is a highly malignant tumor of bone, mostly affecting children and adolescent age groups. This tumor is characterized by complex genetic changes, which are supposed to be involved in osteosarcoma tumorigenesis. Secondary osteosarcoma also can develop as sequences of bone abnormalities such as fibrous dysplasia, Paget’s disease, and osteomyelitis. The peak incidence of osteosarcoma occurs between 1 - 22 years old groups with a smaller peak
after age 50. Osteosarcoma typically arises at the growth plate (Metaphysis) of the long bones, and it is mostly high grade and tends to develop pulmonary metastasis. In spite of clinical management, the patients with metastasis or even with local disease have a poor prognosis. In addition, there is no advance treatment for osteosarcoma, only cytotoxic therapy and limb amputation. This review focuses on the existing data which indicate that osteosarcoma tumors display a broad range of genetic alterations, including loss, deletion, and rearrangements of chromosomal regions. There has evolved some inactivation of tumor suppressor genes and deregulation of some signalling pathways. Certain studies found that p53 and pRb mutations are most commonly reported in osteosarcoma patients and also reported in the animal model cell lines.
This review focuses on the importance of animal models because of the difficulty to obtain human samples with different classifications of the disease, and also a limited number of clinically related with biological insights. Furthermore, human tissue is often taken from chemotherapy patients who then have the problems of
a cellular tumor stroma and tumor necrosis induced by the chemotherapy. For these reasons, relevant animal models are essential to provide an investigational tool for the study of osteosarcoma. This review provides the comparison of human, canine, and murine models in particular, and feline and rabbit in general including incidence, risk factors, clinical presentation and diagnosis, genetic abnormalities, biological behaviors and prognostic factors. Finally, this review
suggests osteosarcoma should be considered as a dedifferentiated disease caused by genetic changes that interrupted osteoblast differentiation from mesenchymal stem cells. Thus, understanding the molecular pathology of osteosarcoma could lead to the development of better diagnosis and prognostic markers, as well as therapeutic agents for osteosarcoma patients.
Original languageEnglish
Article number15
Pages (from-to)50-63
Number of pages14
JournalThe Role of Animal Models in Determining the Genetic Bases of Osteosarcoma
Issue number15
Publication statusPublished - 30 Apr 2013


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