The role of surgery and adjuvants to survival in Pagetic osteosarcoma.

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.
Questions/purposes: 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.