title: The role of surgery and adjuvants to survival in Pagetic osteosarcoma.
abstract: BACKGROUND
  Osteosarcoma is a rare complication of Paget's disease with a very poor prognosis. Treatment is controversial: the older age of the patients affected by Paget's disease may limit the use of chemotherapy and axial involvement may limit the practicality of surgery.
  
  
  OBJECTIVE
  The purposes of this study are (1) to report the survival in patients treated for osteosarcoma in Paget's disease; (2) to identify correlations between type of treatment and survival comparing our data with those in the literature; (3) to determine if the extent of Paget's disease and risk of malignant transformation are associated; (4) to assess if prognosis is related with site; and (5) to identify the variations of histologic subtypes of these osteosarcomas.
  
  
  METHODS
  We retrospectively reviewed the medical records of 26 patients treated between 1961 and 2006 who had bone sarcoma arising from a site of Paget's disease. Twenty two of the 26 patients had surgery. In six surgery only was performed; three had surgery, adjuvant chemotherapy, and radiotherapy; one surgery and radiotherapy; 12 underwent surgery and chemotherapy, adjuvant in 10 patients and neoadjuvant in two; two had only radiotherapy and two had only chemotherapy. We performed survival analyses between various combinations of treatment.
  
  
  RESULTS
  At last followup four patients had no evidence of disease (NED) at a minimum followup of 42.6 months (mean, 139 months; range, 42.6-257.4 months) and 22 died with disease (DWD) at a minimum time of 1 month (mean, 20.2 months; range, 1-84 months). One of the six patients (11%) treated with surgery only had NED at 10 years; the other five died from disease at a mean of 30 months. Three of 12 patients (25%) treated with surgery and chemotherapy are NED at a mean followup of 12 years; nine died of disease at a mean of 24 months. All patients treated without surgery died at a mean of 7.5 months (range, 1-13.7 months).
  
  
  CONCLUSIONS
  Despite improvements in surgery and medical treatments the prognosis remains poor in patients with Paget's sarcoma.
authors.affiliation: Department of Orthopedics, Istituto Ortopedico Rizzoli and University of Bologna, Via Pupilli 1, Bologna, Emilia Romagna, 40136, Italy. pietro.ruggieri@ior.it
authors.forename: Pietro
authors.initials: P
authors.lastname: Ruggieri
authors.forename: Teresa
authors.initials: T
authors.lastname: Calabrò
authors.forename: Maurizio
authors.initials: M
authors.lastname: Montalti
authors.forename: Mario
authors.initials: M
authors.lastname: Mercuri
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000671:Amputation, Surgical; D001859:Bone Neoplasms / Q000175:diagnosis / Q000209:etiology / Q000401:mortality / Q000601:surgery / Q000628:therapy*; D017024:Chemotherapy, Adjuvant; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D007558:Italy; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D020360:Neoadjuvant Therapy; D019637:Orthopedic Procedures / Q000009:adverse effects; D010001:Osteitis Deformans / Q000150:complications* / Q000175:diagnosis; D012516:Osteosarcoma / Q000175:diagnosis / Q000209:etiology / Q000401:mortality / Q000601:surgery / Q000628:therapy*; D018714:Radiotherapy, Adjuvant; D012189:Retrospective Studies; D015996:Survival Rate; D013997:Time Factors; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome