Sarcomatous change in the Pagetoid tibiae

Abstract

We reviewed 13 cases of tibial Paget's sarcoma constituting 14% of all registered Paget's sarcoma cases of the Scottish Bone Tumour Registry between January 1947 and June 2004. Eleven patients were male, and in ten patients the tumour involved the upper half of the tibia. In all cases, the main presenting feature was progressively worsening pain followed by a mass in six, a pathological fracture in five and tibial bowing in four. In nine patients, a lytic lesion was seen radiologically. Histologically, there were three osteosarcomas and ten malignant fibrous histiocytomas. Limb ablation was carried out in 11 patients, of whom nine had trans-femoral amputation. Six patients received adjuvant radiotherapy and/or chemotherapy. Post-operative complications included stump revision in two cases, non-union of a pathological fracture of the tibial tuberosity and a stress fracture. The median survival was 17 months.

Paget's disease of bone

“…Bisphosphonates are often effective at helping bone pain but it is uncertain at present if they alter the natural history of Paget's or prevent complications. The prognosis is poor for patients who develop osteosarcoma, even with agressive treatment (Sharma et al, 2005).…”

Current options for the treatment of Paget’s disease of the bone

“…Bisphosphonate treatment is frequently given before elective orthopedic surgery to try to reduce operative blood loss, but the effects of bisphosphonate therapy on blood loss have never been studied in a controlled trial. Orthopedic surgery might also be needed in patients who develop osteosarcoma, but the prognosis is poor even with aggressive operative treatment, with an overall 5-year survival of about 6% 112–115…”