Sarcomatous change in the Pagetoid tibiae

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.