A REVIEW OF 29 CASES OF PATHOLOGICAL FRACTURES ASSOCIATED WITH PAGET’S SARCOMA

Abstract

This study aimed to analyse clinical, radiological and histological features of 29 cases of pathological fractures of the long bones either presented as or occurred during the course of sarcomatous degeneration in Paget’s disease of bone utilising Scottish Bone Tumour Registry between 1950 to 2000. The mean age was 67.2 (range, 53–81) years with 16 males and 13 females. The commonest bone affected was the femur (n=13) followed by humeral (n=8), tibial (n=5), ilium (n=2) and os calcis (n=1) fracture. The mean duration of symptoms prior to presentation was two months. Fourteen patients presented with pain associated with a pathological fracture. One patient presented with painless lump and eleven with painful swelling followed by a pathological fracture. In the remaining three, the patients reported with a delayed or non-union of the pathological fracture. Nine patients presented acutely as pathological fracture being their main presenting symptom. The typical radiological appearance was a lytic (n=22). Histology showed predominantly osteosarcoma (n=17) followed by malignant fibrous histiocytoma (n=7) and pleomorphic sarcoma (n=5). Amputation was carried out in 16, internal fixation in 5 and replacement arthroplasty in two (1-cemented bipolar and 1-Charnley’s total hip arthroplasty). In addition, adjuvant radiotherapy in 13, adjuvant chemotherapy in 3 and combined radiotherapy-chemotherapy in 2 patients was instituted. The mean survival was 19.3 months (humeral=4.6, pelvifemoral=30, tibiocalcaneal=23.3 months). In conclusion, the majority of the pathological fractures in Paget’s sarcoma behave differently from their counterparts (non-Pagetic pathological fractures) with regard to their limb ablation treatment rather than stabilisation operation. The actual occurrence of a pathological fracture did not show to significantly affect the overall survivorship.