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.

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.

Fig. 1A–C

Osteosarcoma involving the right calcaneus. (A) The first radiograph shows a lytic lesion of the bone. (B) Bone scan shows increased uptake of the calcaneus. (C) CT scan shows osteolysis of the Pagetic calcaneus with disruption of the posterior cortical.

Fig. 2A–C

Distal femur reconstruction after resection for osteosarcoma in Paget’s disease of bone. (A) Osteolytic disruption by osteosarcoma developed a pathologic fracture of the anterior femoral cortex. (B) Coronal MRI view shows intramedullary extension and involvement of the adjacent soft tissues. (C) Radiograph shows reconstruction with a GMRS® prosthesis (Stryker, Howmedica, Osteonics, Allendale, NJ).

Fig. 3

Kaplan-Meier curve of all 26 patients shows a 5-year survival rate less than 25%. There is a higher mortality rate in the first 2 years after treatment.

Fig. 4

Kaplan-Meier curve reporting the type of treatment outlines the high mortality in patients treated without surgery. The curve shows a better trend (p = 0.1057) in patients treated with surgery only or with surgery plus chemotherapy (CHT).

Fig. 5

Kaplan-Meier curve compares survival of patients with pulmonary metastatic disease with survival of patients who are lung tumor-free. Patients with pulmonary metastatic disease at the time of diagnosis had worse survival (p = 0.013) than those who did not.