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PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
3-4
pubmed:dateCreated
2008-6-3
pubmed:abstractText
Purpose Paget' s osteosarcoma has a fearful reputation with a quoted survival of at best 5% at 5 years.We therefore reviewed our experience of 26 patients treated over the last 25 years using modern staging and limb salvage techniques to see if there had been any improvement in survival. Subjects: We identified 26 patients on the Royal Orthopaedic Hospital Oncological database with a diagnosis of sarcoma secondary to Paget's disease.Results: The survival rate was 53% at 1 year, 25% at 2 years and no patient survived for 5 years.The median survival was 21 months for those treated with curative intent and 7 months for those treated palliatively. Four of the five patients treated with limb-sparing surgery developed local recurrence between 5 and 12 months, the fifth died at 14 months.There was no difference in survival between amputation and limb salvage.Discussion: The development of sarcomatous change in Paget's disease is well recognised. It represents an important segment of primary bone tumours in patients 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 similarly disappointing with no survivors at 5 years despite modern methods of management of bone tumours.While there is no difference in local recurrence rates or survival between limb reconstruction and limb ablation the poor prognosis for both means that neither can be recommended at present. Sarcomatous change in Pagetoid bone should therefore be regarded as a different disease to primary osteosarcoma. It remains an incurable disease with a poor prognosis.
pubmed:commentsCorrections
pubmed:language
eng
pubmed:journal
pubmed:status
PubMed-not-MEDLINE
pubmed:issn
1357-714X
pubmed:author
pubmed:issnType
Print
pubmed:volume
3
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
191-2
pubmed:year
1999
pubmed:articleTitle
Paget's Osteosarcoma - no Cure in Sight.
pubmed:affiliation
The Royal Orthopaedic Hospital Oncology Service Bristol Road South Birmingham B 31 2AP UK.
pubmed:publicationType
Journal Article