Bone sarcoma complicating Paget's disease.<i>A report of 3 cases with long survival</i>

Schatzki, Stefan C.; Dudley, H. Robert

doi:10.1002/1097-0142(199005/06)14:3<517::aid-cncr2820140311>3.0.co;2-5

Cited by 20 publications

(2 citation statements)

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“…In his illustrations of the yeoman, Sir Paget portrayed “a cancerous growth … forming around the radius.” Eventually, he would describe osteosarcoma developing in 5 of the 23 patients with Paget's disease that he followed (2) . Since that time, numerous papers have reported an increased risk of bone sarcomas in Paget's disease, so that by 1951, a total of 102 cases of sarcomatous degeneration of pagetic bone had been collected (1,3–34) …”

Section: Introductionmentioning

confidence: 99%

Osteosarcoma in Paget's Disease of Bone

Hansen

Seton

Merchant

2006

Journal of Bone and Mineral Research

166

110

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Paget's disease of bone (PDB) is a focal disorder of bone metabolism first described by Sir James Paget in 1876. It is presumed benign in nature and mediated by abnormal osteoclast function. The incidence of osteosarcomas complicating PDB is estimated at <1%. These cancers occur mostly in persons with long-standing, polyostotic disease and affect patients in their seventh decade or when osteosarcoma is remarkably rare in the general population. Epidemiological studies suggest that this late peak of osteosarcomas is absent in regions where Paget's is infrequently reported. Whereas PDB has a predilection for the axial skeleton, skull, femurs, and tibias, pagetic osteosarcoma tend to spare the spine, and are reported more commonly in the pelvis, femur, humerus, and skull. A molecular basis for the association of osteosarcoma with Paget's disease is unclear. These osteosarcomas are osteogenic in origin, consistently arise in sites of pagetic bone, and may present as metachronous, multifocal lesions. On histopathology, the lesions are usually osteoblastic, and the tumor phenotype is sometimes characterized as an exaggerated, chaotic form of the accelerated bone remodeling that characterizes PDB. New insights from the biology of adolescent osteosarcomas, VCP and SQSTM1 mutations now defined in patients with Paget's disease, and emerging evidence that stromal lesions are present in patients with Paget's disease are changing the way we think about the pathogenesis of PDB and the rare complication of pagetic osteosarcomas.

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Section: Introductionmentioning

confidence: 99%

Osteosarcoma in Paget's Disease of Bone

Hansen

Seton

Merchant

2006

Journal of Bone and Mineral Research

166

110

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“…Complications of Paget's disease include skeletal deformities, fractures, left ventricular failure, and cranial nerve impairment [29,32]. Bone sarcoma is a rare complication of Paget's disease [2,33,35] reportedly occurring in 0.1% to 0.95% of patients [20,27,37].…”

Section: Introductionmentioning

confidence: 99%