Paget's Sarcoma

     Paget's disease of bone is characterized by an excessive and abnormal remodeling of bone. Paget's sarcoma is a rare form of osteosarcoma that occurs in about 5% of patients with advanced polyostotic Paget's disease. Most patients are 55-80 years old. The most common sites of malignant transformation are the femur, pelvis, humerus and craniofacial bones in that order. Paget's sarcoma has a very poor prognosis, with 8-10% survival at 5 years and a mean survival of one year'. Factors contributing to mortality include the aggressive and high grade anaplastic tumors, the poor health of the
age group in which it occurs, surgically inaccessible tumor sites, and the increased vascularity of the bone that occurs with Paget's disease which predisposes to hematological metastatses (especially to the lungs). Other tumors that occur in Paget's disease include malignant fibrous hystiocytomas and
chondrosarcomas.
     Paget's sarcoma usually presents as a new, progressive pain in a patient with long standing Paget's disease. Other symptoms can include soft tissue swelling or pathological fractures. The serum alkaline phosphatase which is elevated with Paget's disease may rise further with onset of sarcoma.
     Patients often complain of pain before the tumor can be easily seen on a plain radiography and the diagnosis can often be delayed. Eventually, cortical destruction and an ill defined tumor mass extending into the soft tissue with a background of Paget's disease become apparent. If one Paget's
sarcoma is found, the entire body should be surveyed as the tumor often arises in more than one site.
     The exact pathology of lesions arising from Paget's disease need to be confirmed by biopsy. The tumor cells of Paget's sarcoma are derived from osteoblasts, have large, pleomorphic nuclei, are poorly differentiated and produce osteoid. The tumor can have different appearances depending on the
matrix produced: bone (osteoblastic), fibrous (fibroblastic), cartilage (chondroblastic) or blood vessels (telangiectatic). Periosteal and juxtacortical sarcomas do not arise from Paget's disease.
     Treatment for Paget's sarcoma depends on the tumor stage and the general status of the patient, and can vary from wide resection and chemotherapy to palliative radiation for pain control.

Sources
Bullough, Peter, Orthopaedic Pathology (third edition), Times Mirror International Publishers Limited: London, 1997.

Huvos, Andrew, Bone Tumors: Diagnosis. Treatment and Prognosis, W.B. Saunders Co., 1991.

Smith, J., Yeh S., Bone Sarcomas in Paget's Disease: A Study of 85 Patients, Radiology, 152:583, 1984.

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