Patients with mutations in the retinoblastoma gene have a several hundred fold greater incidence of osteosarcoma.
As with most malignancies, the precise pathogenesis is unclear.
The disease is associated with mutations in the retinoblastoma (Rb) gene, and deletions of or mutations in the p53 gene.
Other conditions that are associated with high bone turnover, such as Paget's disease and fibrous dysplasia of bone predispose to the development of osteosarcoma.
The tumor commonly arises during the time of maximal bone growth (late adolescence).,
Most common primary malignant tumor of bone.
More commonly seen in men (about 2:1).
|General Gross Description|
Arise from the metaphysis of long bones.
Often big, bulky tumors.
Tan-white and gritty with areas of necrosis, hemorrhage and cystic degeneration.
The tumor frequently grows through the cortical bone and raises the periosteum.
The triangular space thus generated between the raised periosteum and the edge of the bone can be seen radiographically.
This triangle is often referred to as the Codman's triangle.
|General Microscopic Description|
The histologic feature pathognomonic of osteosarcoma is the production of neoplastic osteoid matrix.
Tumor cells are osteocytes or osteoblasts and resemble normal osteoblasts. However, bizarre cells and atypia are invariably seen.
Other types of matrices, such as cartilage, are frequently seen.
Cotran RS, Kumar V, Robbins SL: Robbins Pathologic Basis of Disease. 5th edition. Philadelphia, W.B. Saunders, 1994, pp. 1234.
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||Synopsis by: T.V. Rajan M.D.Ph.D. (T1X500M91803)