Surgical management of neoplastic complications of Paget's disease.

Pagetic sarcoma is a rare anaplastic malignancy with a peak incidence in the seventh and eighth decades of life; it usually occurs in patients with polyostotic Paget's disease. The most common tumor type is osteosarcoma. In one-third of the cases, presentation is a spontaneous pathologic fracture of an affected long bone. Amputation is the most appropriate form of surgical management in most cases because of the aggressive behavior of the sarcoma and its usually late presentation in this elderly population. However, selected patients with extremity lesions may be managed by pre- and postoperative chemotherapy and wide curative resection with limb salvage reconstruction. It is essential to differentiate pagetic sarcoma from metastatic carcinoma in pagetic bone and from a benign giant cell tumor.