Paget's Osteosarcoma — no Cure in Sight

[摘要] PurposePaget' s osteosarcoma has a fearful reputation with a quotedsurvival of at best 5% at 5 years.We therefore reviewed our experience of 26 patients treatedover the last 25 years using modern staging and limb salvage techniques to see if there hadbeen any improvement in survival.Subjects:We identified 26 patients on the Royal Orthopaedic Hospital Oncologicaldatabase with a diagnosis of sarcoma secondary to Paget's disease.Results:The survival rate was 53% at 1 year, 25% at 2 years and nopatient survived for 5 years.The median survival was 21 months for those treated withcurative intent and 7 months for those treated palliatively. Four of the five patients treatedwith limb-sparing surgery developed local recurrence between 5 and 12 months, the fifthdied at 14 months.There was no difference in survival between amputation and limbsalvage.Discussion:The development of sarcomatous change in Paget'sdisease is well recognised. It represents an important segment of primary bone tumours inpatients over 40 years of age.The prognosis is appalling. Indeed only 15 of 368 cases (4%)from a number of historical series have survived more than 5 years. Our results are similarlydisappointing with no survivors at 5 years despite modern methods of management of bonetumours.While there is no difference in local recurrence rates or survival between limbreconstruction and limb ablation the poor prognosis for both means that neither can berecommended at present. Sarcomatous change in Pagetoid bone should therefore beregarded as a different disease to primary osteosarcoma. It remains an incurable diseasewith a poor prognosis.

[学科分类] 肿瘤学